Publisher: Mattioli 1885 srl (Total: 9 journals)   [Sort by number of followers]

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Acta Bio Medica     Open Access   (Followers: 1, SJR: 0.224, CiteScore: 1)
Confinia Cephalalgica et Neurologica     Open Access  
Dermatology Practical & Conceptual     Open Access  
European J. of Oncology and Environmental Health     Open Access   (Followers: 8, SJR: 0.112, CiteScore: 0)
La Medicina del Lavoro     Partially Free   (Followers: 1, SJR: 0.299, CiteScore: 1)
Medicina Historica     Open Access  
Mediterranean J. of Hematology and Infectious Diseases     Open Access   (SJR: 0.506, CiteScore: 1)
Progress in Nutrition     Open Access   (Followers: 2, SJR: 0.193, CiteScore: 0)
Sarcoidosis Vasculitis and Diffuse Lung Diseases     Open Access   (Followers: 1, SJR: 0.765, CiteScore: 2)
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Mediterranean Journal of Hematology and Infectious Diseases
Journal Prestige (SJR): 0.506
Citation Impact (citeScore): 1
Number of Followers: 0  

  This is an Open Access Journal Open Access journal
ISSN (Print) 2035-3006
Published by Mattioli 1885 srl Homepage  [9 journals]
  • Samir Ballas Obituary

    • Authors: Giuseppe G.M. Leone
      Abstract: no abstract
      PubDate: Sun, 30 Oct 2022 00:00:00 +000

    • Authors: Elvira Pelosi; Dr. Germana Castelli, Ugo Testa
      Abstract: In spite recent progresses, acute myeloid leukemia (AML) remains a disease associated with poor prognosis, particularly in older AML patients unfit to tolerate intensive chemotherapy treatment. The development and introduction in therapy of the drug Venetoclax, a potent BH3 mimetic targeting the antiaopoptotic protein BCL-2, inducing apoptosis of leukemic cells, has shown to be a promising treatment for newly diagnosed, relapsed and refractory AML patients ineligible for induction chemotherapy. Combination treatments using Ventoclax and a hypomethylating agent (azacytidine or decitabine) or low-intensity chemotherapy have shown in newly diagnosed patients variable response rates, with highly responsive patients with NPM1, IDH1-IDH2, TET2 and RUNX1 mutations and with scarcely responsive patients with FLT3, TP53 and ASXL1 mutations, complex karyotypes and secondary AMLs. Patients with refractory/relapsing disease are less responsive to Venetoclax-based regimens. However, in the majority of patients the responses have only a limited duration and development of resistance is frequently observed. Understanding mechanisms of resistance is of crucial importance for the development of new strategies and identification of rational drug combination regimens. In this context, two strategies seem to be promising: (i) triplet therapies based on the combined administration of Venetoclax, a hypomethylating agent (or low-dose chemotherapy) and an agent targeting a specific genetic alteration of leukemic cells (i.e., FLT3 inhibitors in FLT3-mutated AMLs) or an altered signaling pathway; (ii) combination therapies based on the administration of two BH3 mimetics (i.e., BCL-2 +MCL-1 mimetics) and a hypomethylating agent.
      PubDate: Sat, 29 Oct 2022 00:00:00 +000

    • Authors: İbrahim Halil Açar; Sebnem Izmir Güner , Muzeyyen Aslaner Ak, Mesut Gocer, Erman Ozturk , Figen Atalay, Gulden Sincan , Aysun Senturk Yikilmaz, Omer Ekinci, Idris Ince, Emine Gulturk , Nazli Demir, Ali Dogan, Yildiz Ipek, Birol Guvenc
      Abstract: Objectives: Patients with hematologic malignancies have a high risk of coronavirus disease 2019 (COVID-19) mortality. This study aimed to investigate the impact of COVID-19 on mortality rates in patients with various hematologic malignancies and to determine risk factors associated with all-cause mortality. Methods: A multi-center, observational retrospective analysis of patients with hematologic malignancies infected with COVID-19 between July 2020 and December 2021 was performed. Demographic data, clinical characteristics, and laboratory parameters were recorded. Patients were grouped as non-survivors and survivors. All-cause mortality was the primary outcome of the study. Results: There were 569 patients with a median age of 59 years. Non-Hodgkin lymphoma (22.0%) and multiple myelomas (18.1%) were the two most frequent hematologic malignancies. The all-cause mortality rate was 29.3%. The highest mortality rates were seen in patients with acute myeloid leukemia (44.3%), acute lymphoid leukemia (40.5%), and non-Hodgkin lymphoma (36.8%). The non-survivors were significantly older (p<0.001) and had more comorbidities (p<0.05). There were significantly more patients with low lymphocyte percentage (p<0.001), thrombocytopenia (p<0.001), and high CRP (p<0.001) in the non-survived patients. Cardiac comorbidities, (p=0.016), cytotoxic chemotherapy (p=0.024), low lymphocyte percentage (p=0.025), thrombocytopenia (p<0.0001), and high CRP values (p=0.017) were the independent risk factors for the prediction of mortality. Conclusions: In patients with hematologic malignancies, coexistent COVID-19 leads to a higher mortality rate in elderly patients with more comorbidities. Acute myeloid and lymphoid leukemia and non-Hodgkin lymphoma have the highest mortality rates. Cardiac diseases, cytotoxic chemotherapy, lymphopenia, thrombocytopenia, and high CRP are the independent risk factors for mortality in hematologic malignancy patients with COVID-19. Keywords: Covid; Hematologic Malignancy; Cytotoxic chemotherapy.
      PubDate: Sat, 29 Oct 2022 00:00:00 +000
  • KIR2DL2, KIR2DL5A and KIR2DL5B genes induce susceptibility to dengue virus
           infection while KIR3DL3 and KIR2DS5 confers protection

    • Authors: Aziz Sidi Aristide TAPSOBA; Florencia Wendkuuni Djigma, Bagora BAYALA, Pegdwendé Abel SORGHO, Lassina TRAORE, Théodora Mahoukèdè ZOHONCON, Shoukrat Ohuwa Toyin BELLO, Prosper BADO, Bapio Valérie Elvira Jean Télesphore BAZIE, Fiffou YOUGBARE, Marius Ayaovi SETOR, Esther Mah Alima TRAORE, Dorcas OBIRI-YEBOAH, Albert Théophane YONLI, Jacques SIMPORE
      Abstract: Background/objective: Dengue, an emerging and re-emerging viral disease, is a major public health problem. The aim of this study was to investigate the influence of KIRs genes polymorphism and KIRs genotypes in susceptibility of dengue virus infection and disease severity in a population from Burkina Faso through a case-control study.Methods: KIRs genes determination were performed using PCR-SSP in 50 patients infected by dengue virus (DENV) and 54 Healthy controls (HC) subjects which never having infected by DENV.Results: Data analysis showed significant association between frequencies of three KIR genes and dengue virus infection (DF): KIR2DL2 (OR: 7.32; IC: 2.87-18.65; P < 0.001); KIR2DL5A (OR: 15.00, IC: 5.68-39.59; P < 0.001) and KIR2DL5B (OR: 11.43; IC: 4.42-29; P < 0.001). While, KIR3DL3 (OR: 0.13, IC: 0.052-0.32; P < 0.001) and KIR2DS5 (OR: 0.12; IC:  0.04-0.30; P < 0.001) were associated with protection against DF.  KIR2DL4 (OR: 9.75; IC95%: 1.33-70.97; p: 0.03) and KIRD3DL1 (OR: 12.00; IC95%: 1.60-90.13; p: 0.02) were associated with an increased risk in the development of secondary dengue infection (SDI).Conclusion: The results suggest a contribution of KIR2DL2, KIR2DL5A and KIR2DL5B genes in the susceptibility of DF development. While, KIR3DL3 and KIR2DS5 were found to be associated with protection against DF development by enhancing both the innate and acquired immune responses.
      PubDate: Sat, 29 Oct 2022 00:00:00 +000

    • Authors: Peng Peng; Fengming XU, Jixing YI, Bumin LIANG, Cheng TANG, Qing FENG
      Abstract: Objective: To explore the relationship between the liver T2* values of thalassemia patients measured by Circle Cardiovascular Imaging CVI42 (CVI42), CMRtools / Thalassemia Tools (CMRtools) and Excel spreadsheet (Excel), and the three software's accuracy in clinical grading of liver iron concentration (LIC). Methods: The liver T2* images thalassemia patients were imported into CVI42 and CMRtools to measure the T2* value. And the signal intensity (SI) of the T2* image, measured in the MR scanning equipment, was input into Excel to calculate T2* value. The relationship of the T2* values measured by the above three software were compared. And LIC clinical grading was performed on the three measured T2* value results, and the LIC (LICF) provided by FerriScan was used as the reference standard to compare the accuracy of the three grading results. Results: There was no statistical difference between the T2* values measured by CVI42, CMRtools and Excel (P>0.05), but there was a high degree of consistency between them (P<0.05), and there was a high linear positive correlation between the them (P<0.05). There was no statistical difference between the LIC grading results of CVI42 and CMRtools and the LICF grading results (P>0.05). There is a significant difference between the LIC grading results of Excel and the LICF grading results (P<0.05). Conclusion: The liver T2* values measured by CVI42, CMRtools and Excel are equivalent. However, CVI42 and CMRtools have better diagnostic accuracy for LIC clinical grade, while Excel has worse diagnostic accuracy. Keywords: THALASSEMIA; lIVER T2 VALUE;
      PubDate: Sat, 29 Oct 2022 00:00:00 +000
           COVID-19 PATIENTS

    • Authors: HADI YASSINE
      Abstract: Background: The heterogeneity of coronavirus disease of 2019 (COVID-19) lies within its diverse symptoms and severity, ranging from mild to lethal. Acute respiratory distress syndrome (ARDS) has been shown to be a leading cause of mortality in COVID-19 patients, characterized by a hyper cytokine storm. Autoimmunity is proposed to occur as a result of COVID-19, given the high similarity of the immune responses observed in COVID-19 and autoimmune diseases. Here, we investigate the level of autoimmune antibodies in COVID-19 patients with different severities. Results: Initial screening for antinuclear antibodies (ANA) IgG using ELISA revealed that 1.58% (2/126) and 4% (5/126) of intensive care unit (ICU) COVID-19 cases expressed strong and moderate ANA levels, respectively. An additional sample was positive with immunofluorescence assays (IFA) screening. However, all the non-ICU cases (n=273) were ANA negative using both assays. Samples positive for ANA were further confirmed with large-scale autoantibody screening by phage immunoprecipitation-sequencing (PhIP-Seq). The majority of the ANA-positive samples showed "speckled" ANA pattern by microscopy, and revealed autoantibody specificities that predominantly targeted proteins involved in intracellular signal transduction, metabolism, apoptotic processes, and cell death by PhIP-Seq; further denoting reactivity to nuclear and cytoplasmic antigens. Conclusion: Our results further support the notion of routine screening for autoimmune responses in COVID-19 patients, which might help improve disease prognosis and patient management. Further, results provide compelling evidence that ANA-positive individuals should be excluded from being donors for convalescent plasma therapy in the context of COVID-19.
      PubDate: Sat, 29 Oct 2022 00:00:00 +000
  • The possible role of chronic infection in the etiopathogenesis of a case
           of 5q-syndrome associated with tuberculosis and abnormality of the X

    • Authors: Lubomir Mitev
      PubDate: Sat, 29 Oct 2022 00:00:00 +000
  • Monocyte HLA-Dr Expression to Monitor Immune Response and Potential
           Infection Risks Following Vaso-Occlusive Crises in Patients with Sickle
           Cell Anemia

    • Authors: Romain Fort; Guillaume Monneret, Elie Nader, Giovanna Cannas, Philippe Connes, Fabienne Venet, Arnaud Hot
      Abstract: No abstract required for a scientific letter
      PubDate: Sat, 29 Oct 2022 00:00:00 +000
  • Covid-19; Cholera and Crimean-Congo hemorrhagic fever in Iraq: A country
           with three outbreaks

    • Authors: Nawfal R Hussein
      Abstract: NA
      PubDate: Sat, 29 Oct 2022 00:00:00 +000

    • Authors: Cristina Papayannidis; Vincenzo Federico, Luana Fianchi, Patrizia Pregno, Novella Pugliese, Alessandra Romano, Federica Irene Grifoni
      Abstract: Systemic mastocytosis (SM) is a rare disease with a range of clinical presentations, and the vast majority of patients have a KIT D816V mutation that results in gain of function. The multikinase/KIT inhibitor midostaurin inhibits the D816V mutant, and has a well-established role in treatment of advanced SM. Even if considered the standard of therapy, some open questions remain on how to optimize the management of midostaurin in daily practice. The current review presents the opinions of a group of experts who met to discuss routine practice with the use of midostaurin in patients with advanced SM. The efficacy and safety of midostaurin in Phase 2 trials are overviewed, followed by practical guidance for optimal management of therapy and adverse events during therapy with midostaurin. Specific guidance is given for initiating therapy and evaluating response with midostaurin in terms of general assessment and laboratory, instrumental, pathological, and molecular exams. Special consideration is given to dose interruption, reduction, and discontinuation of therapy as well as adverse event management and supportive therapy. Patients should be informed about possible side effects and receive not only practical advice to avoid or limit them, but also antiemetic prophylaxis so that therapy with midostaurin can continue as long as clinical benefit is observed or until unacceptable toxicity occurs. Lastly, considerations on the use of midostaurin during the ongoing Covid-19 pandemic are made. The overall scope is to provide guidance that can be useful in daily practice for clinicians using midostaurin to treat patients with advanced SM.
      PubDate: Sat, 29 Oct 2022 00:00:00 +000

    • Authors: Hong Li; Kehong Bi, Saran Feng, Yan Wang, Chuansheng Zhu
      Abstract: Background: MiR-129 promotes the chemosensitivity of cancer cells in many cancers. The role of miR-129 in acute myeloid leukaemia (AML) is unclear. We predicted that premature miR-129 might interact with circEHBP1, a well-characterized oncogene in bladder cancer. We then analyzed the interaction between circEHBP1 and miR-129 in AML. Methods: Expression of circEHBP1 and miR-129 in AML patients) was determined by RT-qPCR before and after treatment with adriamycin (ADR. Detection of circEHBP1 was performed with cellular fractionation assay in nuclear and cytoplasm samples of AML cells. The direct interaction between circEHBP1 and premature miR-129 was explored with an RNA-RNA pulldown assay. Finally, overexpression assays followed by RT-qPCRs were performed to analyze the role of circEHBP1 in the maturation of miR-129. Results: Compared to controls, AML patients exhibited increased expression of circEHBP1 and premature miR-129 but decreased mature miR-129. Altered gene expression was more obvious in ADR resistant group than in the sensitive group. CircEHBP1 was detected in both nuclear and cytoplasm and directly interacted with premature miR-129. Overexpression of circEHBP1 increased the level of premature miR-129 but decreased the level of mature miR-129. In AML cells, circEHBP1 suppressed ADR-induced cell apoptosis and suppressed the enhancing effects of miR-129 on cell apoptosis. More importantly, the role of circEHBP1 in regulating cell apoptosis is more obvious in ADR resistance cells. Conclusion: Therefore, circEHBP1 may suppress the maturation of miR-129 to increase the chemoresistance of cancer cells to ADR in AML. Keywords: acute myeloid leukaemia, circEHBP1, miR-129, adriamycin
      PubDate: Sat, 27 Aug 2022 00:00:00 +000

    • Authors: Saiduo; Wei; Jichan, Xinchun, Hongye, Ning, Xiangao Jiang
      Abstract: To understand the clinical and imaging manifestations and the treatment and follow-up of hepatic tuberculosis (HTB), we retrospectively analyzed the clinical and imaging data of 29 patients with HTB who had been diagnosed clinically or by biopsy, and the clinical and imaging data had been summarised. Patient characteristics were followed up after anti-TB drug treatment. The median age of the 29 patients with HTB was 37 years, and most were male (58.6%). The patient's symptoms included fever (48.2%), respiratory symptoms (27.5%), abdominal pain (24.1%), and abdominal distension (10.3%). Elevated erythrocyte sedimentation rate (79.3%), elevated serum C-reactive protein (75.8%) and hypoalbuminemia (62.0%) were common features. Three patients were serologically positive for acquired human immunodeficiency syndrome, and two were serologically positive for hepatitis B surface antigen with normal tumor markers. The 29 patients with HTB included 17 with serous HTB, 9 with parenchymal HTB (8 with parenchymal nodular HTB and 1 with parenchymal miliary HTB), 1 with intrahepatic abscess type HTB, and 2 with hilar HTB. Approximately 86% of the patients also had pulmonary TB. Most of the serous HTB patients also had tuberculous peritonitis. Enhanced computerized tomography scans of the serous and parenchymal HTB cases showed the progressive development of lesions. Abnormal blood perfusion was observed in the hepatic artery, and the clearest evidence of TB was observed in the hepatic portal vein. Magnetic resonance imaging indicated that the lesions returned a high signal in the diffusion-weighted imaging sequence. However, the lesions' apparent diffusion coefficient values reflected high signals. The Xpert MTB/RIF test detected Mycobacterium TB complex in the liver biopsy fluid from 10 patients. Regarding histopathology, one patient showed granulomatous inflammation, and one patient's acid-fast bacillus (AFB) stain was positive. The treatment of two patients was stopped due to their adverse reactions to the drugs and the risk of creating drug-resistant TB. The remaining patients received anti-TB treatment, but one subsequently died, and two were unavailable for follow-up. The clinical symptoms of HTB are difficult to detect, and it has diverse manifestations by imaging, with no obvious specificity in terms of pathological results. Therefore, follow-up of liver lesions for checking anti-TB therapy is another method for diagnosing HTB. In addition, early active anti-TB treatment can achieve good curative results. Keywords: hepatic tuberculosis; CT; clinical features; X-PERT; imaging diagnosis
      PubDate: Sat, 27 Aug 2022 00:00:00 +000

    • Authors: Indira Jaxybayeva; Riza Boranbayeva, Sagira Abdrakhmanova, Raikhan Maitbassova, Pakhitkanym Ishuova, Dinagul Bayesheva, Nurila Maltabarova, Adyl Katarbayev, Kumisgul Umesheva, Tatyana Marshalkina, Lyazat Manzhuova, Gulnara Abdilova1, Gulshat Alimkhanova, Gulmira Yerzhanova, Gulnara Bulabaeva, Nazgul Zhanuzakova, Svetlana Anokhina, Aigul Kuatbayeva, Gulnara Tashenova
      Abstract: Background and Objectives: Data with more severe mutations of the SARS-CoV-2 virus, compared with the original wild-type strain of COVID-19 disease, were reported worldwide. The study aims to describe the clinical and laboratory manifestations of a multisystem inflammatory syndrome in children (MIS-C) associated with SARS-CoV-2 in the Republic of Kazakhstan and to compare the severity of the disease depending on the time of the circulating variant of SARS-CoV-2 virus. Material and methods: A retrospective, multicentre, nationwide study of 89 children with MIS-C who received inpatient treatment from August 1, 2020, to December 1, 2021. The patients were allocated into two groups: 1(2020) - 45 children and 2 (2021) - 44 children. Study periods were characterized by the circulation of different strains of the SARS-CoV-2 virus. Results: In children with MIS-C in 2021, acute renal failure, disseminated intravascular coagulation syndrome, and shock were statistically more frequently found, which led to fairly common admittance to the intensive care unit. When comparing laboratory data, the children with MIS-C in 2021 had higher values ​​of inflammation markers: ferritin, procalcitonin, erythrocyte sedimentation rate, leukocytes, and neutrophils. Furthermore, these children had a lower level of lymphocytes than children with MIS-C in 2020. Conclusions: MIS-C is a severe, life-threatening systemic disease characterized by multiple organ damage and important inflammatory changes in laboratory parameters. A more aggressive clinical course of MIS-C in 2021 may be associated with the emergence of new SARS-CoV-2 strains. Keywords: children, MIS-C, variants of SARS-CoV-2
      PubDate: Sat, 27 Aug 2022 00:00:00 +000

    • Authors: guangliang Chen; Dou-dou Li, Sufen Sufen, Shiyu Jiang, Qunling Zhang, jia jin, Zuguang Xia, Yizhen Liu, Xiaojian Liu, Yanzhe Zhu, Yu Chen, Lingli Gu, Xiaonan Hong, Junning Cao, Rong Tao, Fangfang Lv
      Abstract: Background Primary breast diffuse large B-cell lymphoma (PB-DLBCL) is a rare subtype of non-Hodgkin lymphoma (NHL) with limited data on the clinical features and prognostic factors. Patients and Methods A consecutive cohort of patients with PB-DLBCL was retrospectively analyzed in our hospital from February 1997 through July 2018. The primary endpoint is overall survival (OS) contributing to any cause. Results A total of 76 patients were diagnosed with PB-DLBCL. The median age at diagnosis was 51 years (range: 25-80 years), with female prevalence (98.7%). Forty (52.6%) patients had right-sided breast involvement but no bilateral breast involvement at diagnosis. Overall, disease stages IE and IIE were seen in 55 (72.4%) and 21 (27.6%) patients, respectively. According to the stage-modified International Prognostic Index (IPI), 37 (48.7%) patients were classified in the very good risk group (IPI 0). Of the 72 patients available, the non-germinal center B-cell (non-GCB) subtype of DLBCL was observed in 66 (91.6%) patients. All patients received anthracycline-based chemotherapy, 56 (73.7%) with rituximab, 31 (40.8%) also with additional radiation therapy, and 14 (18.4%) patients received a prophylactic intrathecal injection. Seven (9.2%) patients had refractory disease. With a median follow-up of 6.8 years (range 0.4-25.0 years), 10 (13.2%) patients had a relapse in the central nervous system (CNS) site. The 5-year and 10-year OS of all the patients was 97.2% (95% CI: 99.3-89.5) and 84.8% (95% CI: 70.0-93.5), respectively. The median OS was not reached. The median progression-free survival (PFS) was 10.3 years for patients with PB-DLBCL(Figure 2B). The 5-year PFS of all the patients was 76.3% (95% CI: 64.6-84.6). Univariate analysis revealed several prognostic factors, including stage-modified IPI, breast surgery, refractory disease, and CNS relapse. Multivariate analyses produced two independent prognostic factors for patients with PB-DLBCL, including stage-modified IPI score (2-3 versus 0) (hazard ratio: 19.114, 95% CI 1.841 to 198.451, p=0.013) and CNS relapse (hazard ratio: 5.522, 95% CI 1.059 to 28.788, p=0.043). Conclusion In our cohort, PB-DLBCL clinical features are similar to prior literature reports. Stage-modified IPI score and CNS relapse were associated with overall survival.
      PubDate: Sat, 27 Aug 2022 00:00:00 +000
  • Utility of flow cytometry ascitic fluid analysis for rapid diagnosis of
           B-cell peritoneal lymphomatosis

    • Authors: Maria Laura Bisegna; Niccolò Noccioli, Irene Della Starza, Alice Di Rocco, Nadia Peragine, Maria Stefania De Propris
      PubDate: Sat, 27 Aug 2022 00:00:00 +000
  • Congenital Atypical Microcytic Anemia Accompanied by Iron Deficiency and

    • Authors: mustafa özay; zafer bıçakçı
      PubDate: Sat, 27 Aug 2022 00:00:00 +000
  • Bacillus cereus in the neutropenic patient: case report in a patient with
           myelodysplastic syndrome and review of the literature

    • Authors: Mariam Markouli; Sevastianos Chatzidavid, Dimitra Vlachopoulou, Nefeli Giannakopoulou, Amalia Anastasopoulou, Nora-Athina Viniou, Panagiotis Diamantopoulos
      Abstract: Bacillus cereus is a Gram-positive rod that can cause food intoxication, localized infection, or bacteremia with potential organ involvement (e.g., meningitis, cerebral and liver abscesses, pneumonia, etc.). Patients with neutropenia and/or hematological diseases are at greater risk for invasive B. cereus infections.
      PubDate: Sat, 27 Aug 2022 00:00:00 +000
           IN CHILDREN

    • Authors: Davide Pata; Danilo Buonsenso, Piero Valentini
      Abstract: Background and Objectives. Coronavirus disease 2019 (COVID-19) is caused by SARS-CoV-2 and has a clinical presentation ranging from asymptomatic course to flu-like syndrome up to respiratory failure. Seasonal Influenza, due by the influenza viruses and very common in children, can cause symptoms similar to COVID-19. In order to identify clinical and laboratory characteristics that allow health-care workers to differentiate COVID-19 from Influenza, we performed a systematic review of the existing literature in the pediatric age. Methods. The research was done via PubMed for articles published from March 2020 to October 2021, combining the MeSH words "COVID-19" and "Influenza" and "Children" and considering the suggestions of PRISMA Group. Results. The most frequently described symptoms were fever and cough in both groups. In most studies, high fever, cough, nasal congestion or rhinorrhea, vomiting and muscle pain were detected more frequently in the Influenza group. Regarding the value of laboratory tests, the results were mixed. Almost all studies reported significantly lower levels of C-reactive protein and procalcitonin in the COVID-19 group than in the Influenza group. In most manuscripts, COVID-19 had a milder course than Influenza. Conclusions. There are no symptoms characteristic of a single infectious agent, with flu-like disorders being the most common. In addition, laboratory tests do not help in the differential diagnosis but show a limited inflammatory response in COVID-19. This could explain the fewer complications compared to adulthood, with a less severe clinical course. Keywords: COVID-19; Influenza; children
      PubDate: Sat, 27 Aug 2022 00:00:00 +000

    • Authors: Ugo Testa; Dr. Germana Castelli, Dr. Elvira Pelosi
      Abstract: Hematopoietic stem cells (HSCs) ensure the coordinated and balanced production of all hematopoietic cell types throughout life. Aging is associated with a gradual decline of the self-renewal and regenerative potential of HSCs and with the development of clonal hematopoiesis. Clonal hematopoiesis of indeterminate potential (CHIP) is a term defining the clonal expansion of genetically variant hematopoietic cells bearing one or more gene mutations and/or structural variants (such as copy number alterations).  CHIP increases exponentially with age and is associated with cancers, including hematologic neoplasia, cardiovascular and other diseases. The presence of CHIP consistently increases the risk of hematologic malignancy, particularly in individuals who have CHIP in association with peripheral blood cytopenia.   Key words: hematopoiesis, hematopoietic stem cells, clonal hematopoiesis, gene mutations, next generation sequencing.    
      PubDate: Sat, 27 Aug 2022 00:00:00 +000
  • Molecular characteristics of hepatitis B virus in children of Huzhou area,

    • Authors: Fang Jin; Fuchu Qian, Dongli Li, Chenxin Yan, Weihua Zou
      Abstract: Objective: The HBV genotypes and surface gene mutation among children born after universal HBV vaccination program were not been clearly investigated in Huzhou, China. Thus, the present study was to investigate HBV genotypes and surface gene mutation in vaccinated children in this area. Methods: Serum samples were collected from 58 children who were chronic HBV infected in Huzhou. The surface gene of HBV were amplified and direct sequencing. Then, the mutations were analyzed. Results: Fifty-six sample were sequenced successfully. The genotype of HBV was genotype B in 45 children and genotype C in 11 children, respectively. Major serotype was adw (42), followed by adr (11) and ayw (3). Sequence analysis showed that 18 amino acid (AA) substitutions sites in major hydrophilic region (MHR) were identified in children (37.5%, 21/56). Most of the substitutions were associated with vaccine immune escape. The genotypes B had a high mutations frequency in the second loop than genotype C. Of note, eight children with low level anti-HBs were still infected with HBV. Conclusion: The predominant genotype and serotype were B and adw among children in Huzhou. Genotype B, MHR mutations, and low anti-HBs levels might be related to immunization failure, although the large-scare vaccination program remarkably decreased the infection of HBV in children, strengthen immunization is recommend in children with low level anti-HBs.
      PubDate: Sat, 27 Aug 2022 00:00:00 +000
  • Acquired Sideroblastic Anemia: An exploratory Comparative Statistical
           Analysis Between Clonal and Non-clonal cases

    • Authors: Dina Sameh Soliman; Samah Kohla, Shehab Fareed, Susanna Akiki , Aliaa Amer, Ibrahim Ganwo, Prem Chandra, Halima El-Omri, Feryal Ibrahim
      Abstract: Sideroblastic anemia (SA) is a rare heterogenous group of inherited and acquired bone marrow disorders. We retrospectively studied the clinicopathologic characteristics, cytogenetic findings, and disease outcome of patients with acquired sideroblastic anemia (ASA) and performed a comparative analysis between clonal and non-clonal cases. 15 patients of ASA were detected: clonal SA (10 cases, 66.7%) including MDS, MDS/MPN, AML and t-MNs with ring sideroblasts) and SA secondary to non-clonal causes (5 cases; 33.3%), including copper deficiency (2 sideroblastic anemia, copper deficiency, non-clonal sideroblastic anemia cases), pyridoxine deficiency diagnosed during pregnancy, and 2 patients with idiopathic SA.Key Keywords: sideroblastic anemia, copper deficiency, non-clonal sideroblastic anemia
      PubDate: Sat, 27 Aug 2022 00:00:00 +000
  • Identification of Alpha Thalassemia, RNF 213 p.R4810K and PROC p.R189W
           among Children with Moyamoya Disease/Syndrome

    • Authors: Lunliya Thampratankul; Yusuke Okuno, Patcharee Komvilaisak, Duangrurdee Wattanasirichaigoon, Nongnuch Sirachainan
      PubDate: Wed, 29 Jun 2022 00:00:00 +000
  • The correlation between ineffective erythropoiesis biomarkers and
           development of extramedullary hematopoiesis in patients with thalassemia

    • Authors: Siriyakorn Chansai; Supawadee Yamsri, Supan Fucharoen, Goonnapa Fucharoen, Nattiya Teawtrakul
      Abstract: Introduction: Extramedullary hematopoiesis (EMH) is one of the main complications in patients with thalassemia in compensation for the underlying ineffective erythropoiesis. This study aimed to evaluate the association between ineffective erythropoiesis biomarkers and EMH in patients with thalassemia. Methods: A cross-sectional study of thalassemia patients aged >18 years old at Srinagarind Hospital was performed. Phosphatidylserine-exposed red blood cells (PS-exposed RBCs) were studied by flow cytometry. The levels of growth differentiation factor-15 (GDF15) and soluble transferrin receptor (sTfR) levels were determined using ELISA. Results: One hundred and thirty-one patients were enrolled. EMH was found in 34 patients (26.0%). EMH was more prevalent among patients with β-thalassemia as compared to those with α-thalassemia (36.4% vs. 4.7%).  The levels of GDF15 and sTfR were different between the two groups and indicated a greater degree of ineffective erythropoiesis in β-thalassemia patients. The levels of PS-exposed RBCs showed a modest correlation with EMH. Advanced age and the PS-exposed RBC levels had a statistical significance associated with EMH. Conclusions: Advanced age and high PS-exposed RBC levels are associated with EMH in thalassemia. PS-exposed RBCs showed a modest correlation with EMH and may be used to predict the development of EMH in thalassemia.
      PubDate: Wed, 29 Jun 2022 00:00:00 +000
  • Prospective, case-control study of serological response after two doses of
           BNT162b2 anti-SARS-CoV-2 mRNA vaccine in transfusion-dependent thalassemic

    • Authors: NICOLA SGHERZA; Stefania Zucano, Angelantonio Vitucci, Antonio Palma, Francesco Tarantini, Daniela Campanale, Luigi Vimercati, Angela Maria Vittoria Larocca, Domenico Visceglie, Amalia Acquafredda, Angelo Ostuni, Daniela Di Gennaro, Carmen Vitucci, Silvio Tafuri, Pellegrino Musto
      PubDate: Wed, 29 Jun 2022 00:00:00 +000

    • Authors: Bedrettin Orhan; Fahir Özkalemkaş, Vildan Özkocaman, Büşra Gürbüz, Tuba Ersal, İbrahim Ethem Pınar, Cumali Yalçın, Ömer Candar, Sinem Çubukçu, Tuba Güllü Koca, Rıdvan Ali
      Abstract: Background and Objective: Perianal pathologies are the most common cause of perianal infections in patients with hematological malignancies. Perianal infection diagnosis in this group of patients is difficult, thus careful anorectal examination is necessary, with imaging modalities. The literature revealed a knowledge gap on the approach of anal pathologies in patients with neutropenia during diagnosis or chemotherapy. This study aimed to examine our institutional data of perianal complications and investigate the relationship among the white blood cell-neutrophil count, perianal lesion, and the type of treatment in patients with hematologic malignancies during the neutropenic period. Methods: Patients with a hematologic malignancy, hospitalized for cytotoxic chemotherapy, complicated by perianal pathology, and documented by at least one imaging method were included in the study. Results: A total of 42 patients were included in the study. The comparison between the groups revealed no statistical significance between the anal abscess formation and the neutrophil count and previous perianal pathology. A statistical significance in favor of acute myeloid leukemia was found between patient diagnosis and anal abscess development. An inverse relationship was found between the number of white blood cells at hospitalization and having an anal pathology operation. It was observed that patients with high white blood cell count were less operated on due to anal pathology. Conclusions: In conclusion, this article has shown that white blood cell count at the time of hospitalization in patients with hematological malignancy, can affect the operation status of patients due to anal pathologies that may occur in the neutropenic period.
      PubDate: Wed, 29 Jun 2022 00:00:00 +000

    • Authors: Dr. Leonid Livshits; Ms. Tal Bilu, Ms. Sari Peretz, Prof. Anna Bogdanova, Prof. Max Gassmann, Dr. Harel Eitam, Prof. Ariel Koren, Prof. Carina Levin
      Abstract: Introduction: The commonly used method for hematocrit detection, by visual examination of microcapillary tube, known as "micro-HCT", is subjective but still remains one of the key sources for false hematocrit evaluation. Analytical automation techniques have increased standardization of RBC indexes detection; however, indirect hematocrit measurements by blood analyzer, the automated HCT, does not correlate well with "micro-HCT" results in patients with hematological pathologies. We aimed to overcome those disadvantages in "micro-HCT" analysis by using "ImageJ", processing software. Methods: 223 blood samples from the "general population" and 19 from sickle cell disease patients were examined in parallel for hematocrit values using the automated HCT, standard "micro-HCT" and "ImageJ" micro-HCT methods. Results: For the "general population" samples, the "ImageJ" values were significantly higher than the corresponding values evaluated by standard "micro-HCT" and automated HCT, except for the 0 to 2 months old newborns, in which the automated HCT results were similar to the "ImageJ" evaluated HCT. Similar to the "general population" cohort, we found significantly higher values measured by "ImageJ" compared to either "micro-HCT" or the automated HCT in SCD patients. Correspondent differences for the MCV and MCHC were also found. Conclusions: This study introduces "micro-HCT" assessment technique using the image-analysis module of "ImageJ" software. This procedure allows overcoming most of the data errors associated with the standard "micro-HCT" evaluation and can replace the use of complicated and expensive automated equipment. The presented results may be also used to develop new standards for calculations of hematocrit and associated parameters for routine clinical practice.
      PubDate: Wed, 29 Jun 2022 00:00:00 +000

    • Authors: ANTONIO LEONE; Nicola Carlo BIANCO, Giulia D'AMBRA, Salvatore LUCCHESI, Elisa LA ROSA, Amato INFANTE, Daniele PERLA, Consolato GULLI'
      Abstract: Purpose: To evaluate the role of progressive radiographic changes to diagnose diabetic foot osteomyelitis. Materials and Methods: A retrospective review of databases of our Institution was performed to identify all long-standing diabetic foot patients who underwent two radiographic examinations spaced no more than five weeks apart and a subsequent magnetic resonance (MR) examination from November 2015 to November 2020. A total of 46 patients (32 men, 14 women; mean age, 57.3 years) were identified. Results: serial radiographs showed 89% sensitivity, 38% specificity, 80% diagnostic accuracy, 87% positive predictive value (PPV), 43% negative predictive value (NPV) to diagnose osteomyelitis (P value < 0,05).  Bone destruction was the most reliable radiographic sign with 89% sensitivity, 88% specificity, 89% diagnostic accuracy, 97% PPV, 64% NPV (P value < 0,05). Conclusion: Progressive bony changes detected by serial radiographs are a useful tool to diagnose diabetic foot osteomyelitis.
      PubDate: Wed, 29 Jun 2022 00:00:00 +000
           OF CCND1- MEDIATED SOX11

    • Authors: Gang Huang; Jianjun Liao, Mingli Wang, Yali Huang, Mingjie Tang, Yanyan Hao
      Abstract: Mantle cell lymphoma (MCL) is an aggressive lymphoid malignancy with a poor prognosis. Ubiquitin-specific peptidase 9, X-linked (USP9X), has been found to be associated with multiple physiological pathways and regulate a variety of cellular activities. In this study, we explored the role of USP9X in MCL in vitro and in vivo. USP9X was verified to be increased in peripheral blood mononuclear cells (PBMCs) of MCL patients and MCL cells. Moreover, USP9X overexpression and knockdown were performed in MCL cells. We proved that USP9X overexpression promoted proliferation and cell cycle, and suppressed cell apoptosis in MCL cells. Unregulation of angiogenesis and cell migrate were induced by USP9X overexpression in MCL cells. However, USP9X knockdown showed opposite effects. In addition, USP9X was discovered to decrease Cyclin D1 (CCND1)-mediated SOX11 expression in MCL cells. We demonstrated that SOX11 overexpression reversed USP9X knockdown-mediated angiogenesis in MCL cells. Besides, tumor formation was inhibited by USP9X knockdown in mice in vivo. In conclusion, these results revealed that USP9X promoted tumor angiogenesis in MCL via increasing CCND1- mediated SOX11.
      PubDate: Wed, 29 Jun 2022 00:00:00 +000

    • Authors: Elisabetta Schiaroli; Giuseppe Vittorio De Socio, Anna Gidari, Lisa Malincarne, Sara Benedetti , Alessandra Lanzi, Sabrina Bastianelli, Sara Pierucci, Chiara busti, Daniela Francisci
      Abstract: Background and Objective: In patients with mild-to-moderate COVID-19 and at high risk of progression, casirivimab/imdevimab and bamlanivimab/etesivimab were utilized in Umbria from late April to November 2021. This period was characterized by an initial prevalence of alpha (B1.1.1.7) and its progressive substitution with the delta variant (B1.617.2). Many delta infections occurred in patients already recently vaccinated. Our study aimed to observe the clinical outcome of patients treated with mAbs associations in a subgroup in which viral isolation was obtained, the pre and post-infusion neutralizing antibody activity against their viral isolate. Methods: In this retrospective observational study, the clinical outcome before and 30 days after infusion, the baseline neutralizing activity of sera against their viral isolate, and the titers of neutralizing antibodies (NAbTs) one-hour post-infusion relative to the type of mAbs associations were evaluated. Results: Better efficacy of the mAbs combinations relative to monotherapy regarding global hospitalization (p = 0.021) and 30 days symptoms (p<0.001) were seen. Infections after vaccination mostly occurred in the absence of neutralizing antibody titers (NAbT). SARS-CoV-2 delta variants were isolated within 2-4 months from vaccinations without NAbTs, or in the presence of high specific neutralizing activity after 5-6 months. NAbTs were higher after casirivimab/imdevimab infusion (p=0.001). Conclusions: Alpha infections occurred prevalently in unvaccinated patients or after 5-6 months, while delta infections prevailed in vaccinated ones. A poor neutralizing activity in most of these patients was seen. A higher NAbT after infusion of casirivimab/imdevimab was observed.
      PubDate: Wed, 29 Jun 2022 00:00:00 +000

    • Authors: Derya Alabaz; Fadime Eroğlu, Hüseyin Elçi, Ümmühan Çay
      Abstract: Background; Protozoa of the genus Leishmania are obligate intracellular parasites and Leishmania species cause a spectrum of species-specific clinical symptoms known as cutaneous, mucocutaneous and visceral leishmaniasis. Leishmania major and Leishmania tropica are known to cause cutaneous leishmaniasis, while Leishmania infantum and Leishmania donovani cause visceral leishmaniasis (VL). However, molecular studies in recent years have shown that Leishmania species cause different clinical symptoms. Objectives; Our aim is to evaluate the relationship between clinical and molecular characterization of leishmania isolates in children with VL defined in Turkey, which is an intercontinental transitional region. Methods; The clinical diagnosis of VL was confirmed by the detection of amastigotes in the bone marrow aspirate and/or the rK39 test and/or molecular methods (genus specific PCR, Real-Time PCR, ITS1 PCR-RFLP, DNA sequencing). Results: Most of the VL patients were referred from the districts of Adana (53.3%) and others from neighboring provinces; Hatay (16.6%), Osmaniye (3%), Gaziantep (3%), Adıyaman (3%) and 20% case were Syrian emigrants. A total of 30 patients with a clinical diagnosis of VL were confirmed by different diagnostic methods. It was found that 93% positive with microscopic examination, 79.1% with rK39 dipstick test, and 60% with genus specific PCR assay in clinical samples. The Leishmania isolates were identified as L. infantum (40%), L. donovani (26.7%) and L. tropica (23.3%) using Real Time PCR, ITS1 PCR-RFLP and DNA sequencing. There was no cutaneous finding in any case in clinical examination. The most common clinical findings were fever (93.3%), splenomegaly (90%), followed by hepatomegaly (76.6%). The most common laboratory finding was thrombocytopenia (86.6%) followed by anemia (70%). Hemophagocytic lympho-histiocytosis was detected in bone marrow aspiration in two of our patients. Since pentavalent antimony salts treatment initially failed in four patients, it was necessary to switch to Liposomal-Amphotericin B treatment and was successfully treated. Conclusions: The presence of L. tropica in VL patients, despite the absence of cutaneous findings in any of the cases, shows that this strain can cause VL, contrary to conventional knowledge. In the Adana province, where this study was carried out, L. infantum from CL cases in previous studies should be taken into account and visceral spread in CL cases and accompanying cutaneous lesions in VL cases should be investigated in detail. Key Words; Visceral leishmaniasis, DNA Sequencing, Leishmania infantum, Leishmania donovani, Leishmania tropica
      PubDate: Wed, 29 Jun 2022 00:00:00 +000

    • Authors: Xuebing Jing; Cong Lu, Chao Song, Qingkun Han
      Abstract:  Background:It was reported that circular RNA (circRNA) circCTNNA1 plays an oncogenic role in colorectal cancer, while its rfole in mantle cell lymphoma (MCL) is unknown. This study aimed to explore the role of circCTNNA1 in MCL. Methods:Samples of B lymphocytes were collected from 56 MCL patients and 56 healthy controls. The expression of circCTNNA1 and miR-34a in these samples were determined by RT-qPCRs. The direct interaction between circCTNNA1 and miR-34a in MCL cells was detected using RNA-RNA pulldown assay. Overexpression assays were performed to study the interactions between circCTNNA1 and miR-34a. Cell proliferation was assessed with BrdU assay. Results:The results showed that circCTNNA1 was upregulated in MCL and high expression levels of circCTNNA1 predicted the poor survival of MCL patients. MiR-34a was downregulated in MCL and inversely correlated with circCTNNA1. CircCTNNA1 was predicted to interact with miR-34a, and the interaction between them was confirmed by RNA pull-down assay. Interestingly, overexpression of circCTNNA1 and miR-34a did not affect the expression of each other. Cell proliferation analysis showed that overexpression of circCTNNA1 reversed the inhibitory effects of overexpression of miR-34a on cell proliferation. Conclusion:Upregulation of circCTNNA1 in MCL predicts poor survival of patients and it may sponge miR-34a to promote cancer cell proliferation.
      PubDate: Wed, 29 Jun 2022 00:00:00 +000

    • Authors: Panagiota Zikidou; Christina Tsigalou, Gregorios Trypsianis, Alexandros Karvelas, Aggelos Tsalkidis, Elpis Mantadakis
      Abstract: Background and Objectives: Iron deficiency (ID) is a major public health problem with high prevalence in early childhood. We assessed the prevalence of anemia, ID, and iron deficiency anemia (IDA) in healthy children of Thrace, Greece, its correlation with dietary factors, and evaluated the diagnostic performance of hematologic and biochemical markers of sideropenia. Patients and Methods: For 202 healthy children 1-5 years old, a questionnaire was filled out describing their nutritional habits during infancy and early childhood. Venous hemograms along with serum ferritin, TIBC, %TS, and CRP were obtained from all studied children. In a subset of 156 children, the concentration of sTfR was also determined. Results: Children with ID and IDA had significantly lower beef consumption than children without sideropenia (p=0.044). Using the WHO cut-off values of Hb <11g/dl and ferritin <12μg/l, the prevalence of anemia, ID, and IDA was 9.41%, 6.44%. and 3.47%, respectively. If Hb <12g/dl and ferritin<18μg/l were used as cut-offs, the prevalence of anemia, ID, and IDA was 26.73%, 16.33%, and 5.94%, respectively. ROC analysis revealed that at ferritin <12μg/l, sTfR had the highest specificity and PPV but the lowest sensitivity for ID (93%, 47.4%, and 69.2%, respectively), while sTfR/Fer index had the highest sensitivity and NPV (100%). Conclusions: The prevalence of ID and IDA in children 1-5 years old in Thrace is like in other developed countries. sTfR and sTfR/Fer index have the highest accuracy to diagnose ID, with the sTfR/Fer index being superior irrespective of the ferritin cut-off value used to define ID.
      PubDate: Wed, 29 Jun 2022 00:00:00 +000

    • Authors: Paul Kambale-Kombi; Roland Marini Djang’eing’a, Jean-Pierre Alworong’a Opara, Jean-Paulin Mbo Mukonkole, Vincent Bours, Dieu-Merci Mbumba Lupaka, Serge Tonen-Wolyec, Lucien Bolukaoto Bome, Charles Kayembe Tshilumba, Salomon Batina-Agasa
      Abstract: Background & objective: Sickle cell disease (SCD) is now a well-established cause of renal damage. In the northeast of the Democratic Republic of Congo (DRC), SCD is common. However, sickle cell nephropathy remains unstudied in this region. Thus, this study aimed to assess renal abnormalities in SCD patients in Kisangani (northeastern DRC). Methods: This cross-sectional study included 98 sickle cell patients selected from six health facilities in Kisangani and 89 healthy non-sickle cell subjects as the control group. Based on a survey form, a clinical examination and biological tests were performed to collect data related to the sex, age, weight, height, blood pressure, serum creatinine, serum uric acid, urinary albumin/creatinine ratio, and hemoglobin phenotype. We used a spectrophotometer to measure serum creatinine and uricemia, the sickle SCAN® device for hemoglobin phenotype, and an automatic multifunction analyzer for urine albumin/creatinine ratio. Data were entered into an Excel file and analyzed on SPSS 20.0. Results: The mean urine albumin-to-creatinine ratio was 11.79±9.03 mg/mmol in SCD patients, significantly higher than in AA (1.69±1.89 mg/mmol) and AS (2.97±4.46 mg/mmol) subjects. The decrease in glomerular filtration rate was more observed in SCD patients with hyperuricemia compared to those with normal uric acid levels. A significantly elevated prevalence of chronic kidney disease was observed among SCD patients (87.8%) compared to 23.8% in AS and 7.7% in AA subjects. Conclusions: This study highlighted that albuminuria and chronic kidney disease are common in SCD patients in Kisangani. More studies are needed to document these complications further. Keywords: Sickle cell disease, prevalence, sickle cell nephropathy, renal abnormalities, Democratic Republic of the Congo, sub-Saharan Africa.
      PubDate: Wed, 29 Jun 2022 00:00:00 +000
  • Nodular lymphocyte-predominant Hodgkin lymphoma in a patient with Familial
           Mediterranean Fever

    • Authors: Marina Belia; Asimina Papanikolaou, Panagiotis Skendros, Theodoros Vassilakopoulos
      PubDate: Wed, 29 Jun 2022 00:00:00 +000
  • Sinopharm Vaccine, SARS-CoV-2 breakthrough infections and

    • Authors: rujittika mungmunpuntipantip; viroj wiwanitkit
      PubDate: Wed, 29 Jun 2022 00:00:00 +000
  • Can Polycythemia Vera evolve from Acute Myeloid Leukemia' A Case
           Report Showing a Simultaneous Minor JAK2 V617F Mutated Clone,

    • Authors: Beatrice Borsellino; Arianna Savi, Maria Rosaria Pascale, Elisa Meddi, Antonio Cristiano, TIZIANA OTTONE, Maria Cristina Rapanotti, Mariadomenica Divona, SERENA TRAVAGLINI, Enrico Attardi, Elisa Buzzatti, Francesco Buccisano, Maria Teresa Voso
      Abstract: Evolution of myeloproliferative neoplasms (MPN) to acute myeloid leukemia (AML) occurs in 2-10% of patients, whereas the reverse path from AML to MPN has been rarely reported. We herein present a 75-year-old woman with AML, in whom a JAK2-V617F positive polycythemia vera (PV) emerged during follow-up, 19 months from end of consolidation treatment. JAK2-V617F mutation screening retrospectively performed by Next Generation Sequencing (NGS) and JAK2 MutaScreen was negative on the bone marrow sample collected at AML diagnosis. However, using droplet digital PCR (ddPCR), we detected a minor JAK2-V617F mutated clone present at AML onset. A TET2 R550 mutated clone persisted at stable levels throughout the disease course. This case shows that a very small MPN clone masked at AML diagnosis may expand after treatment end, and be erroneously interpreted as MPN evolving from AML. Very sensitive techniques as ddPCR may help to unravel the true disease history in these cases.
      PubDate: Wed, 29 Jun 2022 00:00:00 +000
  • The Mediterranean Journal of Hematology and Infectious Diseases: A New
           Milestone and a Hope for Researchers in Third World Countries

    • Authors: Nawfal R Hussein
      PubDate: Thu, 28 Apr 2022 00:00:00 +000
  • Post-therapy B regulatory cells might early predict relapse in Hodgkin

    • Authors: Valentina Giudice; Luca Pezzullo, Giuseppe Ciancia, Matteo D'Addona, Francesca D'Alto, Marisa Gorrese, Bianca Cuffa, Carmine Selleri
      PubDate: Thu, 28 Apr 2022 00:00:00 +000
  • Ixazomib-associated skin exfoliation

    • Authors: Yali Zhang; Long Zhao, Yuancheng Guo, bei liu
      PubDate: Thu, 28 Apr 2022 00:00:00 +000

    • Authors: Prof. Giuseppe Leone; Emiliano Fabiani, Maria Teresa Voso
      Abstract: The aim of our review has been to give an appropriate idea of analogies and differences between primitive MDS (p-MDS) and t-MDS throughout an accurate reviewing of English peer-reviewed literature focusing on clinical, cytogenetic, epigenetic, and somatic mutation features of these two groups of diseases. Therapy-related MDS (t-MDS) are classified by WHO together with therapy-related acute myeloid leukemia (t-AML) in the same group, named therapy-related myeloid neoplasm. However, in clinical practice, the diagnosis of t-MDS is made with the same criteria as for primitive MDS (p-MDS), and the only difference is a previous non-myeloid neoplasm. The prognosis and the consequent therapy can be established following the same criteria as for p-MDS, and the therapy is generally decided using the same criteria. We stress the possible difference in cytogenetics, mutations, and epigenetics to distinguish the two forms. Actually, there is no marker specific for t-MDS either in cytogenetics, epigenetics, or mutations; however, some alterations are also frequent in t-MDS and, in general, they induce a poorer prognosis. So, the high-risk forms in t-MDS are prevalent. The present literature data suggest classifying the t-MDS as a subgroup of MDS and introducing some parameters to evaluate the probability of previous therapy in inducing MDS. An important issue remains the patient’s fitness, which strongly influences the outcome. Keywords: MDS, t-MDS- Cytogenetics, Mutation, Epigenetics.
      PubDate: Thu, 28 Apr 2022 00:00:00 +000

    • Authors: Anna Maria Testi
      Abstract: The past three decades have brought major therapeutic advances in the treatment of acute promyelocytic leukemia (APL) both in adults and children. The current state-of-the-art treatment with all-trans retinoic acid (ATRA) and arsenic trioxide (ATO) in combination or not with chemotherapy results in long-lasting remission and cure in more than 90% of newly diagnosed patients. These treatments have made relapse a rare event. The detection of PML-RARA transcript by polymerase chain reaction (PCR) during treatment and follow-up can predict a hematological relapse. All studies have suggested a survival benefit in patients with molecular relapse given pre-emptive therapy compared with those treated at the time of overt hematological relapse. ATO-based regimens, seem to be effective for the achievement of a second molecular complete remission (CR). Patients in second molecular CR, are generally considered candidates for autologous hematopoietic stem cell transplant (HSCT), while for those with persistent molecular disease, allogeneic HSCT could be offered if a suitable donor is identified. Except for sporadic pediatric reports, most of the evidence for the use of HSCT for treatment of relapsed/refractory APL, comes from adult literature. We hereby provide a review of published pediatric data that evaluated the role of HSCT in children with refractory/recurrent APL disease.     KEYWORDS: acute promyelocytic leukemia; relapse; hematopoietic stem cell transplant; children; adolescents
      PubDate: Thu, 28 Apr 2022 00:00:00 +000

    • Authors: Alessandro Buonomo; Eleonora Nucera, Marianna Criscuolo
      Abstract: Management of Indolent and Smoldering SM is focused on preventing anaphylactic reactions, identifying and avoiding symptom triggers. Skin and gastrointestinal symptoms are managed with H1- and H2-antihistamines. When skin symptoms are not adequately controlled, leukotriene antagonists and oral psoralen combined with ultraviolet therapy may be added. Proton pump inhibitors, sodium cromolyn and oral corticosteroids may be added for gastrointestinal symptoms. Patients should be prescribed with self-injectable epinephrine and trained to treat recurrent cardiovascular symptoms or anaphylaxis. Depression and cognitive impairment require a psychiatric evaluation for tailored treatment. Bone involvement is managed with bisphosphonates, and eventually interferon. Omalizumab is effective on all vasomotor symptoms, including anaphylaxis, but not on respiratory, musculoskeletal, and neuropsychiatric symptoms. A cytoreductive treatment is not recommended unless anti-mediator therapy has failed. Venom immunotherapy is mandatory for patients with hymenottera venom allergy. There is not a curative option for patients with Advanced SM. The available therapeutic options include tirosin-kinase inhibitors and cladribine, with variable duration and extent of response. Imatinib mesylate was the first drug approved for SM lacking the cKIT D816V mutation; dasatinib and nilotinib are not effective. Midostaurin is active on both wild type and mutant cKIT D816V while Avapritinib is a selective cKIT D816V inhibitor: they are approved for treatment of Advanced SM. Cladribine is a purine analogue with significant activity against monocytes that were thought to have a common progenitor with mast cells. Allogeneic stem cell transplantation is usually performed in younger selected patients. MASTOCYTOSIS; THERAPY; SKIN INVOLVEMENT, SYSTEMIC MASTOCYTOSIS; TYROSINKINASEINHIBITORS; MIDOSTAURIN.
      PubDate: Thu, 28 Apr 2022 00:00:00 +000

    • Authors: Abdalla Awidi; Farah Qasem, A'sem Abu-Qamar, Batool Aqel, Rand Aladayleh, Ilham Alteerah, Ahmad Magableh, , Hisham Bawa”neh, Feras Al-fararjeh
      Abstract: Abstract Background and objective: Very scanty reports from the middle east and north Africa (MENA) region have been published on multiple myeloma (MM). Multiple myeloma registry has been established at Jordan University Hospital (JUH) since 2009. In this work we aim to review Multiple Myeloma registry with data from 113 patients who were diagnosed with MM at JUH and analyze their management and course. Methods: This is a non-interventional, and retrospective analysis of MM registry from 2009-2016 involving 113 patients at JUH. Statistical analysis was done using the Statistical Package for the Social Sciences (SPSS). Overall survival (OS) was analyzed with the Kaplan-Meier method. P value was considered significant if it was (<0.05). Results: We found no gender difference in this registry. The median age is 62 years. Most patients are ISS stage II and III (36.28% for each). Immunoglobulin type G Kappa is the dominant subtype. Bone pain is the most common presenting symptom. The most common laboratory finding is anemia (45.6%). Most of our patients (85.2%) had received thalidomide and dexamethasone, while only 14.8% received bortezomib, thalidomide, and dexamethasone. The mean overall survival (OS) in our patients was 74 months, and the median survival was 38 months. Median OS for ISS stage I, II, and III were 96, 46, and 16 months respectively. Conclusion: MM in a developing country presents a challenging disease compared with that in industrial countries in both the epidemiology and management. An improved road map in the care of MM in these countries is needed. The use of three or four drug combination upfront is warranted. However, this is limited because of the high cost of these drugs. We expect the following decade to show better survival and quality of life for MM patients once these drugs are widely used. KEYWORDS: MYELOMA, DEVELOPING COUNTRIES, JORDAN, NEW THERAPIES, THERAPY COST.
      PubDate: Thu, 28 Apr 2022 00:00:00 +000

    • Authors: Karin Mayer; Nicolaus Hegge, Ernst Molitor, Peter Brossart, Corinna Hahn-Ast
      Abstract: Background: In febrile neutropenia linezolid (LIN) and also vancomycin (VAN) can be used if a gram-positive infection is suspected. Interestingly there is no literature in which both are compared in the setting of febrile neutropenia. Therefore we provide here the results of a retrospective analysis of adding VAN versus LIN in patients with febrile neutropenia. Methods: Patients with haematological diseases and febrile neutropenia after myelosuppressive chemotherapy and no clearance of infection after the first empiric broad spectrum antibiotic, which were escalated to VAN or LIN from 03/2010 to 03/2014 at the University Hospital Bonn were included in this retrospective analysis. Results: Out of the 73 patients 50 had received VAN and 23 LIN. The median time of hospitalisation in the LIN cohort was significant shorter than in the VAN cohort (LIN 16 days vs VAN 20 days p=0.046). Successful defervescence with the escalation to VAN or LIN could be detected in 76% of the LIN cases and 50% in the VAN group (p=0.052). This trend to a better efficacy with LIN was also shown by a higher rate of discontinuation of VAN and escalation to another antibiotic scheme (54.2%) than in the LIN cohort (24%, p=0.052). Conclusion: The antibiotic therapy in febrile neutropenia with LIN showed a trend to a better efficacy than therapy with VAN. But because of the small sample size and the retrospective manner VAN may still be considered a reasonable option in neutropenic fever and randomized studies are needed in this field. KEYWORDS: GRAM+ INFECTIONS, VANCOMYCIN, LINEZOLID, DOSE ESCALATION.
      PubDate: Thu, 28 Apr 2022 00:00:00 +000

    • Authors: Ming Tong; Xiquan Yan, Yu Jiang, Zhaoxia Jin, Shengjiao Zhu, Lianhong Zou, Yanjuan Liu, Qing Zheng, Guoqiang Chen, Ruifeng Gui Gui, Zhilan Zhou, Xiaotong Han, Jiangming He, Siqing Yin, Changchun Ma, Wen Xiao, Yong Zeng, Fang Chen, Yimin Zhu
      Abstract: Background: COVID-19 is characterized by endothelial dysfunction and is presumed to have long-term cardiovascular sequelae. In this study, we aimed to explore the serum levels of endothelial biomarkers of COVID-19 recovers 1-year after hospital discharge. Methods: A clinical follow-up study, including 345 COVID-19 survivors from Huanggang, Hubei, and 119 age and gender matched healthy controls were enrolled in the study. A standardized symptom questionnaire, electrocardiogram and Doppler ultrasound of lower extremities, routine blood tests, biochemical and immunological tests were collected, and serum levels of soluble vascular cell adhesion molecule-1(VCAM-1), intercellular cell adhesion molecule-1(ICAM-1), P-selectin, and fractalkine were measured by enzyme-linked immunosorbent assays. Results: 1-year after discharge, 39% of recovers possessed post-COVID syndromes, while no deep vein thrombosis was detected in all screened. No significant differences in circulatory inflammatory markers (leukocytes, neutrophils, lymphocytes, C-reactive protein and interleukin-6), alanine aminotransferase, estimated glomerular filtration rate, glucose, triglycerides, total cholesterol and D-dimer observed among controls and previously mild or severe infected. Furthermore, serum levels of VCAM-1, ICAM-1, P-selectin, and fractalkine presented no significant differences between survivors and healthy controls. Conclusions: SARS-CoV-2 infection may not impose a higher risk of long-term cardiovascular sequelae, even for those recovered from severe illness. KEYWORDS: COVID, ENDOTHELIAL DYSFUNCTION, INFLAMMATORY MARKERS, ADHESION MOLECULES, THROMBOSIS.
      PubDate: Thu, 28 Apr 2022 00:00:00 +000

    • Authors: Yali Zhou; Guiping Liao, Xiaolin Yin, Sheng He, Yi Wu, Jian Xiao, Zhili Geng, Qiuying Huang, Ganghui Luo, Kun Yang
      Abstract: Background: IVS-II-5 G>C (HBB: c.315+5 G>C) is a rare β-thalassemia mutation. However, there is no clear evidence regarding the effect of this defect or co-inheritance of other β-thalassemia mutations on phenotypes. Methods: The clinical phenotypes associated with compound heterozygosity for the IVS-II-5 G>C mutation with other β-thalassemia mutations, together with the potential effect of the genetic modifiers α-thalassemia were studied in 13 patients. Analyses of red cell indices, hemoglobin component, iron status, and α-globin genes were carried out in 19 heterozygotes. Results: Next-generation sequencing of 24 undiagnosed patients with thalassemia major (TM) or thalassemia intermedia (TI) identified 13 carriers of the IVS-II-5 G>C mutation. There was a wide spectrum of phenotypic severity in compound heterozygotes and 6 (46.2%) of 13 were transfusion dependent. Analysis of 19 heterozygotes indicated that most were hematologically normal without appreciable microcytosis or hypochromia, and approximately half had normal hemoglobin A2 levels at the same time. Conclusion: Compound heterozygotes for IVS-II-5 G>C and other severe β-thalassemia mutations are phenotypically severe enough to necessitate appropriate therapy and counseling. Co-inheritance of this nucleotide substitution with other β-thalassemia mutations may account for a considerable portion of the incidence of undiagnosed patients with TI and TM in Guangxi. The IVS-II-5 G>C mutation can pose serious difficulties in screening and counseling. Keywords: β-thalassemia; IVS-II-5 G>C; genotype; phenotype
      PubDate: Thu, 28 Apr 2022 00:00:00 +000

    • Authors: Claire Coutureau; Philippe Nguyen, Maxime Hentzien, Peter Joe Noujaim, Sarah Zerbib, Damien Jolly, Lukshe Kanagaratnam
      Abstract: Background and Objectives Severe forms of SARS-CoV-2 infections are associated with high rates of thromboembolic complications. Professional societies and expert consensus reports have recommended the use of anticoagulants for COVID-19 hospitalized patients. Our study aimed to compare the effect of therapeutic, intermediate and prophylactic doses of heparin on 6-week survival in patients hospitalized for COVID-19.
      The study sample is a French cohort of COVID-19 patients, hospitalized between February 25th and April 30th 2020. Patients were assigned to one of 3 anticoagulation dose groups, based on the maximum dose they received for at least 3 days (prophylactic, intermediate or therapeutic). The main outcome was survival up to 42 days after hospital admission. Multivariate Cox regression models were performed to adjust analyses for confounding factors. Results A total of 323 patients were included. The mean age of the study sample was 71.6 ± 15 years and 56.3% were men. Treatment with the intermediate versus prophylactic dose of anticoagulation
      (HR = 0.50, 95%CI = [0.26 ; 0.99], p = 0.047) and with therapeutic versus prophylactic dose (HR = 0.58 95%CI = [0.34 ; 0.98], p = 0.044) was associated with a significant reduction in 6-week mortality, after adjustment for potential confounding factors. Comparison of therapeutic versus intermediate doses showed no significant difference in survival. Conclusions Our results reported a significant positive effect of intermediate and therapeutic doses of heparin, compared with a prophylactic dose, on 6-week survival for hospitalized COVID-19 patients.
      PubDate: Thu, 28 Apr 2022 00:00:00 +000

    • Authors: Mervat Khorshied; Iman A, Shaheen, Yasmeen M.M Selim, Asmaa O. Elshahawy, Ilham Youssry
      Abstract: Background: Oxidative stress plays a pivotal role in the pathophysiology of sickle cell
      disease (SCD) and its associated disease complications. Superoxide Dismutases (SODs) are
      protective enzymes against oxidative stress. SOD2 deficiency results in the accumulation of
      oxidized red cell proteins, increased rate of hemoglobin oxidation, decreased red cell membrane
      deformability, and subsequently decreased red cells survival.
      Objective: The current study was designed to determine the effect of SOD2 Val16Ala gene
      polymorphism (rs4880) on SOD2 level and their possible impact on SCD disease severity in a
      cohort of Egyptian SCD patients.
      Methods: Genotyping SOD2 Val16Ala polymorphism by TaqMan allelic discrimination assay for
      hundred SCD patients and a hundred age-sex matched healthy controls revealed the genotypic
      and allelic frequencies of the studied polymorphism in the SCD patients were close to that of the
      Results: Serum SOD2 level was significantly lower in those having the polymorphic genotypes
      (p=0.005). SOD2 level inversely correlates with the annual rate of hospitalization (r=-0.023, p=
      Conclusion: SOD2 Val16Ala polymorphism was associated with low serum SOD2 levels that may
      predict disease severity. KEYWORDS. SCD, EGYPT, SOD2, POLYMORPHISM.
      PubDate: Thu, 28 Apr 2022 00:00:00 +000

    • Authors: Cumali Yalçın; Fahir Özkalemkaş, Vildan Özkocaman, Tuba Ersal, İbrahim Ethem Pınar, Bedrettin Orhan, Ömer Candar, Sinem Çubukçu, Tuba Güllü Koca, Merve Nur Akyol, Nevriye Gül Ada, Cüneyt Özakın, Esra Kazak, Halis Akalın, Rıdvan Ali
      Abstract: Background: This study aimed to evaluate the effects of the appropriate use of empiric glycopeptide therapy in hematologic malignancy patients with febrile neutropenia (FN). Materials and Methods: Patients with FN who were hospitalized in our clinic and started empiric glycopeptide therapy were retrospectively analyzed. Empiric glycopeptide treatment initial indications were determined according to 7 specific criteria in the IDSA guidelines. In addition, the duration of glycopeptide use according to initial indications, causative pathogens in culture positivity, frequency of VRE infection, and the mortality rate was identified. Results: 87 patients were included. Of these, 102 episodes of FN were analyzed. Appropriate use of glycopeptides was observed in 98% of patients. The most common initial indication for glycopeptide was skin or soft-tissue infection, with 52% (n = 53). The mean duration of glycopeptide use was 11 (2–22) days. The duration of glycopeptide use was longer in patients with catheter-related infections than in those with severe mucositis and hemodynamic instability (p = 0,041/p = 0,016). The duration of glycopeptide use was shorter in patients with consolidation therapy than those without consolidation therapy. The mortality rate in culture-positive patients was significantly higher than in those who were culture-negative (p = 0.041). When the patients who were culture-positive and culture-negative were compared, there was no significant difference in the duration of glycopeptide use. Conclusion: This study showed that the mortality rate was higher in culture-positive patients. Additionally, the use of glycopeptides should be discontinued early with no evidence of gram-positive infection. KEYWORDS: HEMATOLOGICAL MALIGNANCIES; GRAM+ INFECTION, GLYCOPEPTIDES,
      PubDate: Thu, 28 Apr 2022 00:00:00 +000

    • Authors: Araya Satdhabudha; Chanapai Chaiyakulsil, Paskorn Sritipsukho, Phakatip Sinlapamongkolkul, Utairat Chaumrattanakul, Auchara Tangsathapornpong, Pornumpa Bunjoungmanee, Patcharapa Thaweekula, Amolchaya Kwankuac, Onsuthi Pharadornuwata, Tananya Lokanuwatsatiena, Pichaya Tantiyavarong, Pakarat Pranudomrata, Chatchai Mingmalairak
      Abstract: Background: Few studies had focused on the epidemiological and clinical characteristics of pediatric COVID-19 (SARS-CoV-2) during Delta and pre-Delta eras in Asia, despite it being a pandemic. Objective: To study the epidemiological and clinical characteristics of three waves of pediatric COVID-19 infections in a tertiary-care setting in Thailand. Methods: This retrospective study reviewed all PCR-confirmed pediatric (0-18 years of age) COVID-19 infections between January 13th, 2020 and October 31st, 2021, in a tertiary care system, Thailand. Results: There were 1,019 patients, aged 0.02 - 18 years, median age of 9.2 years, with no gender differences. Asymptomatic cases accounted for 35.7%, of which 18.9% had abnormal chest X-ray findings. The majority of cases were classified as having mild clinical symptoms, with only 0.8 and 0.4% developing severe and critical illness, respectively. There were no deaths. The Delta dominant group appeared more transmissible, but we did not see any difference in disease severity. Upper respiratory tract symptoms were predominant, while few cases had lower respiratory signs. The sensitivity and specificity of dyspnea symptoms to predict pneumonia (abnormal chest X-ray) were 14% and 95%, respectively, with a likelihood ratio 3.37. The overall prognosis was good, with only 0.01 % needing respiratory equipment. All cases showed clinical improvement with a decent recovery. Conclusion: Pediatric COVID-19 during Delta era appeared generally more transmissible but benign. One-fifth of cases had pneumonia, but few cases needed respiratory support. Prevention remains important for disease control. Keywords: Pediatric, COVID-19, SARS-CoV-2, Delta, Epidemiology  
      PubDate: Thu, 28 Apr 2022 00:00:00 +000
  • The venetoclax/azacitidine combination targets the disease clone in Acute
           Myeloid Leukemia, being effective and safe in a patient with COVID

    • Authors: Antonio Cristiano; Raffaele Palmieri, Emiliano Fabiani, Tiziana Ottone, Mariadomenica Divona, Arianna Savi, Francesco Buccisano, Luca Maurillo, Corrado Tarella, William Arcese, Maria Teresa Voso
      PubDate: Thu, 28 Apr 2022 00:00:00 +000
  • Managment of Relapsed/Refractory ALL With Inotuzumab During COVID-19. A
           Casr Report

    • Authors: Martina Di Palma; Elio Gentilini, Chiara Masucci, Alessandra Micozzi, Ombretta Turriziani, Antonino Mulè, Robin Foà, Maurizio Martelli, Gabriella D'Ettorre, Saveria Capria, Sabina Chiaretti
      Abstract: Management of patients with concomitant acute lymphoblastic leukemia (ALL) and COVID-19 infection is challenging. We describe the clinical history of a 40-year-old male with relapsed B-common ALL who developed Sars-CoV2 prior to treatment initiation with inotuzumab. Since the patient was asymptomatic for COVID-19, the first dose of inotuzumab was administered, followed by remdesivir as prophylaxis. However, a worsening in respiratory findings led to a delay in administering the following doses of inotuzumab. Interestingly, even if the patient did not receive the full inotuzumab cycle, he achieved a complete hematologic remission: furthermore, he spontaneously developed anti-sars-COV2 antibodies. COVID-19 treatment also included convalescent plasma, leading to negativization of the viral load. The patient, after COVID-19 recovery, received a second full cycle of inotuzumab, underwent allogeneic transplantation, and is currently in complete hematologic and molecular remission, in good clinical conditions, five months from allograft. Keywords: acute lymphoblastic leukemia, COVID-19, inotuzumab, remdesevir, convalescent plasma
      PubDate: Thu, 28 Apr 2022 00:00:00 +000

    • Authors: Emanuele Focà; Benedetta Fumarola
      Abstract: "IMMUNOLOGICAL EVOLUTION OF A COHORT OF HIV-2 INFECTED PATIENTS: PECULIARITIES OF AN UNDERESTIMATED INFECTION" Human Immunodeficiency Virus type 2 (HIV-2) affects a minority of patients in Italy; nevertheless, the increasing migratory flow from higher prevalence areas led to the spread of this virus into our Country. We evaluate clinical, viro-immunological and therapeutic characteristics of patients with HIV-2 infection and HIV-1/HIV-2 dual-infection and the early treatment impact on overall survival and incidence of AIDS event. Methods: We retrospectively analyzed all HIV-2 and HIV-1/HIV-2 positive patients followed in a large Italian clinic from January 1987 to December 2020. We recorded demographic, viro-immunological, clinical and therapeutics data. We performed a descriptive analysis followed by a longitudinal analysis to explore the factors associated with CD4+ lymphocyte trend; lastly, we studied in a multivariable model the possible predictors of death and AIDS in our cohort. Results: 32 subjects were enrolled, 17 (53%) HIV-2 infected and 15 (46.8%) HIV-1/HIV-2 dual-infected; 12 patients were lost to follow up, while 3 died. We found a lack of HIV-2 viremia in 12/32 subjects (37.5%).Most of the patients at baseline had a good viro-immunological profile with HIV-2 RNA <200 copies/ml and CD4+ lymphocyte >200 cell/mcl.
      PubDate: Sun, 27 Feb 2022 00:00:00 +000

    • Authors: Luca Guarnera; Federico Meconi, Roberto Secchi, Maria Rosaria Pascale, Fabiana Esposito, Annagiulia Zizzari, Vito Mario Rapisarda, Manuela Rizzo, Livio Pupo, Maria Cantonetti
      Abstract: Introduction Gastric Diffuse large B‐cell lymphoma (DLBCL) is the most common extra-nodal site of lymphoma’s involvement (30%-40% of all extranodal lymphomas and 55%-65% of all gastrointestinal lymphomas). However, gastric localizations are also sometimes found in systemic DLBCL. Gastric complications such as bleeding, perforation and stenosis under chemotherapy are well documented.  Methods We retrospectively analyzed 15 patients with newly diagnosed DLBCL with gastrointestinal involvement. Endoscopies were performed in these patients before and after treatment. Treatment consisted in cyclophosphamide low-dose pre-phase chemotherapy before conventional-dose chemotherapy. Results Endoscopy at staging detected ulcers in 12 patients (80%). After low-dose pre-phase chemotherapy GI ulcers healed in 91.6% of cases (1 ulcer detected). After the whole treatment (Low-dose pre-phase + chemotherapy) 9 patients (60%) achieved complete response, 4 patients (26.6%) partial response, 2 (13,3%) patients presented disease progression. The most frequent adverse event was neutropenia (73.3%); the most frequent non hematological adverse event was transaminases elevation (20%). Conclusion Cyclophosphamide low-dose pre-phase chemotherapy resulted a safe and effective way to prevent adverse events in systemic DLBCL with gastrointestinal involvement.
      PubDate: Sun, 27 Feb 2022 00:00:00 +000

    • Authors: Muhamad R. Abdel Hameed; Sherein G. Elgendy, Mohamed A El-Mokhtar, Douaa Sayed, Samar M. Mansour, Abeer M. Darwish
      Abstract: Background: Invasive fungal infections (IFIs) are important cause of mortality in acute myeloid leukemia (AML) patients on treatment with intensive induction chemotherapy. Toll-like receptors, mainly Toll-like receptors 2 and 4 (TLR2 and TLR4), play a considerable role in the host defense against microorganisms.  The expression of TLRs and their association with the occurrence of IFIs in patients with AML remains unclear. The aim of this study was to investigate the associations between the T-lymphocyte expression of TLR2 and TLR4 and the occurrence of IFIs in AML patients treated with intensive induction chemotherapy. Materials and Methods: One hundred twenty two newly diagnosed AML patients were evaluated. The laboratory diagnostic techniques for IFIs include culture, microscopic examination, histopathology, galactomannan assay and PCR. The expressions of TLR2 and TLR4 were analyzed by flow cytometry. The Control group included 20 age and sex-matched individuals. Results: There was a significant increase in the expression of TLR4 in AML patients with IFI compared to healthy controls (p = 0.001). TLR2 and TLR4 expressions increased significantly in AML patients with mixed fungal and bacterial infection compared to healthy controls (p= 0.002 and p=0.001, respectively). Conclusion: TLRs expressions could be important biological markers for the occurrence of IFI in AML patients after intensive induction chemotherapy.   Keywords: Invasive Fungal Infection, TLR2, TLR4, Acute Myeloid Leukemia  
      PubDate: Sun, 27 Feb 2022 00:00:00 +000

    • Authors: Mehran Karimi; Tahereh Zarei, Sezaneh Haghpanah , Azita Azarkeiva, Maryam Naderi , Sara Matin , Asghar Bazrafshan , Zohreh Zahedi , Afshan Shirkavand , Parisa pishdad, Vincenzo De Sanctis
      Abstract: Background:  The ongoing COVID-19 pandemic, caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has led to high morbidity and mortality worldwide. Vaccination against SARS-CoV-2 is a leading strategy to change the course of the COVID-19 pandemic. Aims of study: Our aim was to investigate the efficacy and side effects of Sinopharm vaccine in patients with hemoglobinopathies in Iran, and the frequency of breakthrough infection after a full   course of vaccination. Methods: A multicenter cross-sectional study of 434 patients with hemoglobinopathies (303 β-thalassemia major, 118 β-thalassemia intermedia, and 13 sickle-thalassemia) were conducted from March to July 2021 in IRAN. All patients have received the first dose of China Sinopharm vaccine and received the second dose of the vaccine 28 days apart. Antibody testing: Detection of immunity after vaccination was evaluated by commercial enzyme- linked immunosorbent assay (Pishtazteb ELISA commercial kit), including a surrogate virus neutralization test (sVNT), for detection of SARS-CoV-2 immunoglobulins (IgA, IgM, IgG), total neutralizing antibody (NAb). Results: The mean age of patients was 35.0± 8.5 (from 18 to 70) years and 55.6% were positive for antibody. Overall, 48.2% of the studied population had at least one side effect after vaccination. The most frequent side effects were fever and chills, dizziness, and body pain. A total of 90 (20.7%) vaccinated patients developed breakthrough infections after full Sinopharm vaccination. Disease severity was recorded, and it was classified as mild in 77.8%, moderate in 13.6%, and severe in 7.4% of patients. One 28-year-old woman with β- thalassemia major died eight days after the diagnosis of breakthrough SARS-CoV-2 infection. Discussion: No safety concerns were identified in patients who received two doses of Sinopharm vaccine. Its efficacy was not optimal which might be due to lack of its effect on new variations of virus. However, our data show that it can prevent from severity of COVID-19 infection in patients with hemoglobinopathies. The frequency of breakthrough infections after full Sinopharm vaccination support the evolving dynamic of SARS-CoV-2 variants requiring special challenge, since such infection may represent a risk for vulnerable patients.
      PubDate: Sun, 27 Feb 2022 00:00:00 +000
  • TDT Positive High-Grade Lymphoma with Myc, BCL2 And BCL6 Rearrangements: a
           Review of Diagnosis and Treatment

    • Authors: Aditi Singh; MD, Ishaq Asghar, MD, Laura Kohler, DO, Hosam Hakim, MD, Daniel Snower, MD, Daniel Lebovic, MD
      Abstract: In the modern era, classification of neoplasms not only depends on immunomorphological features but also on specific disease defining genetic events. Translocations/rearrangements of MYC/8q24 locus in combination with BCL-2 or BCL6 translocations (double/triple hit) are considered hallmarks of high-grade B-cell lymphoma (HGBL), a type of aggressive mature B-cell lymphoma. When cases with immature immunophenotype present with these rearrangements, establishment of diagnosis becomes very difficult. We herein report an unusual case of B-cell lymphoma that presented with immature morphology and immunophenotype with triple hit gene rearrangements. This case highlights the difficulty in classifying and appropriately treating these patients. The novel aspect of this case is the treatment and outcome with chimeric antigen receptor, or CAR T-cell therapy. Keywords: high-grade Lymphoma, TDT, MYC, BCL6.
      PubDate: Sun, 27 Feb 2022 00:00:00 +000
  • A Suspected Case of Cerebral Fat Embolism Triggering a Drug-resistant
           Status Epilepticus in a HbS/β⁺-Thalassaemia Patient

    • Authors: Marta Bortolotti; Gianluca Costamagna, Marta Mancarella, Delia Gagliardi, Silvia Lanfranconi, Alessia Marcon, Margherita Migone De Amicis, Nereo Bresolin, Stefania Corti, Giovanna Graziadei
      Abstract: Sickle cell disease (SCD) refers to a group of hereditary disorders associated with clinical manifestations of variable severity. C In particular, Fat Embolism Syndrome, a rare and devastating complication of SCD, may preferentially involve non-homozygous patients and patients with mild disease.
      PubDate: Sun, 27 Feb 2022 00:00:00 +000
  • CAR-T cells microscopic and phenotypic identification in the peripheral

    • Authors: LESESVE
      Abstract: CAR-T cells are a new possibility for care poor prognostic conditions. CAR-T cells injection lead to T chimeric cells circulation into the blood. Here is reported the cytomorphologic and immunophenotypic features of CAR-T cells in the peripheral blood.
      PubDate: Sun, 27 Feb 2022 00:00:00 +000
  • Challenging management of severe differentiation syndrome in pediatric
           acute promyelocytic leukemia treated with ATRA/ATO

    • Authors: Alessandro Molinaro; Daniela Zanta, Maria Luisa Moleti, Fiorina Giona, Valentino Conter, Carmelo Rizzari, Andrea Biondi, Anna Maria Testi
      Abstract: The ATRA/ATO combination treatment of acute promyelocytic leukemia (APL) represents a paradigm of successful targeted and chemotherapy-free treatment in oncology. This therapeutic strategy is aimed at sparing patients from chemotherapy toxicity, while maintaining an excellent survival with a low risk of relapse. Main induction treatment-related complications are differentiation syndrome (DS) and hyperleukocytosis, which is related to DS and its severity. In the period December 2019 – December 2020, 8 children with newly diagnosed APL underwent induction therapy with ATRA/ATO in our center. In patients with WBC≥10x109/L  two doses of Gemtuzumab Ozogamicin (GO) were  added. In case of severe DS or hyperleukocytosis the differentiating agents were discontinued, high dose dexamethasone (DXM) and/or hydroxyurea (HU) were recommended. Five patients presented WBC<10x109/L ; all  developed hyperleukocytosis and three also had DS and were initially treated with HU and DXM; due to unsatisfactory control of the symptoms GO was added in two of them.. One of the three patients with presenting WBC≥10x109/L, developed pseudotumor cerebri and another one DS. The supportive treatment was effective in all cases. Our experience shows that patients, treated with ATRA/ATO only, may develop marked hyperleukocytosis and severe DS, which may be unresponsive to discontinuation of differentiating agents and administration of HU and DXM and may benefit from the use of GO. Adequate intensive support therapy is crucial to rescue patients with severe DS.
      PubDate: Sun, 27 Feb 2022 00:00:00 +000
  • Intracardiac thrombosis in the three-year-old boy with normal left
           ventricle systolic function in MIS-C associated with COVID-19

    • Authors: Stasa Krasic; Sasa Popovic, Ruzica Kravljanac, Sergej Prijic, Vladislav Vukomanovic
      PubDate: Sun, 27 Feb 2022 00:00:00 +000

    • Authors: Ilaria Cozzi; Giovanni Rossi, Emma Rullo, Valeria Ascoli
      Abstract: Primary effusion lymphoma (PEL) is a large B-cell lymphoma growing within body-cavities caused by the Kaposi sarcoma-associated herpesvirus (KSHV)/human herpesvirus-8 (KSHV/HHV-8). It is mainly reported in HIV-infected patients. The uncommon occurrence in the elderly supports a form paralleling classic Kaposi sarcoma (KS), i.e. classic PEL, whose characteristics are relatively underexplored. To better understand the diagnostic modalities and clinical-epidemiological features of classic PEL, articles reporting cases of PEL were identified through MEDLINE/EMBASE databases (January 1998-July 2020) and screened according to PRISMA guidelines to extract individual-level data. A comparison was also performed between classic PEL and classic KS to evaluate similarities and differences. We identified 105 subjects (median age 77 years; 86% males), mainly from Mediterranean countries (52%, first Italy) and Eastern Europe (7%). Common comorbidities were heart failure (32%), cirrhosis (16%), and malignancy (20%) including lymphoid neoplasms. Pleural cavity was the commonest site (67%). PEL diagnosis was based on cytomorphology (89%), evidence of KSHV/HHV-8 infection (94%), EBV co-infection (28%) and clonality of IGH (59%), IGK (14%), TRG (9%) alone or in multiple combinations. Compared to KS, age (P<.001), gender-ratio (P=.08) and mortality (P<.001) were significantly higher in PEL, whereas the frequency of PEL as a second primary was similar (P=.44). This is the first systematic review of classic PEL case reports highlighting heterogeneity and lack of a uniform multidisciplinary approach at diagnosis, in the absence of specific guidelines as it happens for rare cancers. It is conceivable that classic PEL is still underdiagnosed in Mediterranean countries wherein KSHV/HHV-8 is endemic.
      PubDate: Sun, 27 Feb 2022 00:00:00 +000

    • Authors: Flavio De Maio; Delia Mercedes Bianco, Giovanni Delogu
      Abstract: Since the emergence of Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) at the end of 2019, a number of medications have been used to treat the infection and the related Coronavirus disease – 19 (COVID-19). Some of the administered drugs were tested or used in practice only on the basis of biological plausibility; a promising strategy was to target the host immune response, with host directed therapies (HDTs), to reduce systemic hyperinflammation and hypercytokinemia responsible for additional tissue damage. We summarize the treatments against SARS-CoV-2 and underline their possible effects on Mycobacterium tuberculosis (Mtb) infection. Both SARS-CoV-2 and Mtb respiratory infections impair the host’s immune response. Furthermore, little research has been conducted on the impact of medicaments used to counteract COVID-19 disease in patients with Latent Tuberculosis Infection (LTBI). A number of these drugs may modulate host immune response by modifying LTBI dynamic equilibrium, favoring either the host or the bacteria. Keywords: SARS-CoV-2, Mycobacterium tuberculosis, COVID-19, Tuberculosis, Host directed therapies
      PubDate: Sun, 27 Feb 2022 00:00:00 +000

    • Authors: Antonio Giordano; Livio Pagano
      Abstract: Cutaneous T-cell lymphomas are a heterogenous group of T-cell neoplasms involving the skin, the majority of which may be classified as Mycosis Fungoides (MF) or Sézary Syndrome (SS). Mycosis fungoides (MF) is usually associated with an indolent clinical course and intermittent, stable, or slow progression of the lesions. Extracutaneous involvement (lymph nodes, blood, or less commonly other organs) or large cell transformation (LCT) may be seen in advanced-stage disease. Sezary syndrome (SS) is a rare leukemic subtype of CTCL characterized by significant blood involvement, erythroderma, and often lymphadenopathy. Although early-stage disease can be effectively treated predominantly with skin-directed therapies, systemic therapy is often necessary for the treatment of advanced-stage disease. Systemic therapy options have evolved in recent years with the approval of novel agents such as vorinostat, brentuximab vedotin, and mogamulizumab. This review aims to discuss the diagnosis and management  of advanced-stages MF and SS.
      PubDate: Sun, 27 Feb 2022 00:00:00 +000
  • Improving BNT162b2 mRNA vaccine tolerability without efficacy loss by
           Pidotimod supplementation

    • Authors: Claudio Ucciferri; Antonio Auricchio, Jacopo Vecchiet, Katia Falasca
      Abstract: Background and objectives: A new pandemic has emerged across the world:Covid-19. Covid-19 has affected hundreds of millions of people globally. To stop the spread of the virus and gain a mass immunity several vaccines have been developed. BNT162b2-mRNA-vaccine has been shown to be largely effective and is widely administered. However the vaccine is not free from adverse events that could discourage vaccination in many people. The aim was evaluated adverse effect and immunological effect after second dose of  BNT162b2-mRNA-vaccine in a healthcare population that take Pidotimod versus control subjects. Methods: All nurses and doctors working in Covid-19 unit were proposed to participate, up to the enrollment of 30participants. 10 participants took Pidotimod 800mg bid orally fasting, from the fourth day before the second dose of the BNT162b2-mRNA-vaccine, for a total of six days. The remaining 20 participants did not take any therapy. We studied the differences in anti-SARS-CoV2 IgM and IgG levels and the difference of frequency of adverse events between the two groups  Results: Although there was no significant difference in IgG production between the two groups, we found a lower frequency of vaccine adverse events in the group supplemented with pidotimod. Conclusions: This work demonstrates how pidotimod, despite not having a proven efficacy in increasing the production of antibodies, significantly reduces the adverse events described compared to people vaccinated without pidotimod supplementation. The results we described in this paper could encourage many more clinicians and people to vaccinate and gain the mass immunity needed to end this pandemic.
      PubDate: Sun, 27 Feb 2022 00:00:00 +000
  • Treating relapsed/refractory acute myeloid leukemia with chidamide,
           fludarabine, cytarabine and granulocyte-colony stimulating factor with
           subsequent bridging to myeloablative allogeneic hematopoietic stem cell

    • Authors: Wen Yao; Xinchen Fang, Peng Jiang, Juan Tong, Liangquan Geng, Xiaoyu Zhu, Baolin Tang, Xiang Wan, Kaidi Song, Lei Zhang, Ping Qiang, Guangyu Sun, Yongsheng Han, Huilan Liu, Zimin Sun
      PubDate: Sun, 27 Feb 2022 00:00:00 +000

    • Authors: XiaoTian Zhang; Yanhui Yu, Chao Zhang, Lijuan Zhao, Hongrui Wang, Guoqing Wang, Ming Yang
      Abstract: A large number of studies have shown that patients with Coronavirus disease 2019 (COVID-19) have different degrees of liver injury. However, the mechanisms of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) invasion into the liver are still not fully understood. This review mainly summarizes the recently published works on the abnormal liver biochemical indicators and the mechanism of viral invasion with liver injury in COVID-19 patients. Generally, SARS-CoV-2 infection of the liver was caused by blood circulation or retrograde infection of digestive tract, which led to the liver injury through direct cytopathic effect induced by virus or immunopathological effect caused by excessive inflammation. Besides these, hypoxia, endothelial injury and drug-induced jury were also the main reasons of liver injury in COVID-19 patients. In the liver function indicators, elevated alanine aminotransferase, aspartate aminotransferase, alkaline phosphatase, gamma-glutamyl transpeptidase, and lactate dehydrogenase levels with reduced albumin levels were observed in COVID-19 patients.
      PubDate: Sat, 01 Jan 2022 00:00:00 +000

    • Authors: Donato Rigante; Elena Rossi, Antonietta Curatola, Giovanna Capozio, Luca Benacquista, Ludovica Iezzi, Donato Rigante
      Abstract: A disparate group of rare hematological diseases characterized by impaired maturation of neutrophil granulocytes defines congenital neutropenias. Neutropenic patients are prone to recurrent infections beginning in the first months of life. Of interest is “cyclic neutropenia”, an ultra-rare disorder revealed by sinusoidal variations of the neutrophil count and periodically-recurring infections every 21 days. Diagnosis of these disorders is frequently obscured by the multiple causes of recurrent fevers in children. Aim of this overview is to outline the physical assessment of children presenting with early-onset symptomatic neutropenia, identify the disease between the many medical conditions and even emergencies which should enter in differential diagnosis, hint at the potential management with granulocyte-colony stimulating factor, define the risk of evolution to hematologic malignancy, and summarize inter-professional team strategies for improving care coordination and outcomes of such patients.
      PubDate: Sat, 01 Jan 2022 00:00:00 +000

    • Authors: Federico Mercolini; Simone Cesaro
      Abstract: SARS-CoV-2 pandemic affected less children and adolescents, morbidity and mortality figures being inferior to that reported for adults. In this review we focused on the clinical course, risk factors for severe COVID-19, mortality, treatment options and prevention measures in the pediatric and adolescent setting with special attention to the pediatric oncohematological patients. In this subgroups of patients, SARS-CoV-2 infection was often asyntomatic but 47 to 68% of patients require hospitalization and 9-10% of those hospitalized needed intensive care with a COVID-19 attributable mortality of about 4%. The multisystem inflammatory syndrome associated to Coronavirus 2019 was less frequent than that reported in the non-oncohematological pediatric population. Noteworthy, the course of COVID-19 was more severe in low-middle income countries. The key measures to prevent SARS-CoV-2 infection are the reduction of patients exposure to the SARS-CoV-2 and vaccination, now available fore care givers and parents and for patients and siblings > 12 years old. The treatment of COVID-19 in pediatric patients was mainly based on supportive care with dexamethasone and heparin prophylaxis for severely ill patients. Other measures, such as convalescent plasma, remdesivir and monoclonal antibodies have been used in limited case or within experimental protocols. Further studies are needed on the risk factors and outcome of SARS-CoV-2 infection in the pediatric immunocompromised patients. 
      PubDate: Sat, 01 Jan 2022 00:00:00 +000
  • The impact of the SARS-CoV-2 pandemic on healthcare provision in Italy to
           non-COVID patients: a systematic review

    • Authors: Annarita Botta; Gianmarco Lugli, Matteo Maria Ottaviani, Guido Ascione, Alessandro Bruschi, Federico Cagnazzo, Lorenzo Zammarchi, Paola Romagnani, tommaso portaluri
      Abstract: Background Italy has been one of the countries most affected by the SARS-CoV-2 pandemic and the regional healthcare system has had to quickly adapt its organization to meet the needs of infected patients. This has led to a drastic change in the routine management of non-communicable diseases with a potential long-term impact on patient health care. We investigated the management of non-COVID-19 patients across all medical specialties in Italy. Methods A PRISMA guideline-based systematic review of the literature was performed using PubMed, Embase, and Scopus, restricting the search to the main outbreak period in Italy (from 20 February to 22 June, 2020). We selected articles in English or Italian that detailed changes in the Italian hospital care for non-COVID-19 patients due to the pandemic. Our keywords included all medical specialties in combination with our geographical focus (Italy) and COVID-19. Results Of the 4643 potentially eligible studies identified by the search, 247 studies were included. A decrease in the management of emergencies in non-COVID patients was found together with an increase in mortality. Similarly, non-deferrable conditions met a tendency toward decreased diagnosis. All specialties have been affected by the reorganization of healthcare provision in the hub-and-spoke system and have benefited from telemedicine.   Conclusions Our work highlights the changes taking place in the Italian public healthcare system to tackle the developing health crisis due to the COVID-19 pandemic. The findings of our review may be useful to analyze future directions for the healthcare system in the case of new pandemic scenarios.  
      PubDate: Sat, 01 Jan 2022 00:00:00 +000

    • Authors: FASOLA ATINUKE
      Abstract: Background: Sickle cell disease is a protean disease with limited data on the phenotypic and genetic variants in Nigeria. This study was conducted to provide baseline data on these variants by characterizing the existing forms of sickle cell disease and correlating these with basic hematological parameters. Methods: Adult and pediatric patients with SCD were recruited from a tertiary health centre in Nigeria. Patients were age and sex matched with healthy controls. Blood samples were obtained for Full Blood Count, phenotyping by High Performance Liquid Chromatography and genotyping for alpha thalassemia by multiplex gap polymerase chain reaction. Data analysis was done using IBM SPSS statistics version 23. Results: A total of 130 patients with sickle cell disease and 117 controls were studied. Alpha thalassemia in the study population was due to a 3.7kb deletion in the alpha globin gene cluster at a prevalence of 45.4% in the patients and 47% in controls. The prevalence of the various existing forms of SCD genotype was: Homozygous S without alpha gene deletion (HbSS)- 39.2%; HbSC - 10.8%; HbSα+1- 35.4%; HbSα+2 - 6.9% and HbSF- 7.7%. HbA2 was significantly elevated in individuals with two alpha gene deletions (HbSα+2). HbF and HbA2 were negatively correlated with each other (r= -0.587, p < 0.001). Individuals with the HbSC genotype followed by HbSα+2 had the best hematological parameters. Conclusions: Hematological parameters varied with hemoglobin genotype. The C hemoglobin and homozygous alpha thalassemia deletion had better ameliorating effect on SCD hematological parameters than the F hemoglobin in this population.  
      PubDate: Sat, 01 Jan 2022 00:00:00 +000

    • Authors: Madeha Abdalla Sayed; Mohamed Abdelhakeem
      Abstract: Back ground: A novel coronavirus which is identified as cause of pandemic situation inFebruary2020 and affecting adult and children with variable presentation and outcome. Objective: We studied the typical and atypical clinical and laboratory presentation of COVID-19 during the peak of the first wave   in two main  referral hospitals, upper Egypt El Minya governorate. Methods:  Among 88 children with suspected cases  tested for COVID-19, only 22 who proved to be  positive. Studied patients were classified into 3 groups based on age. The first group 2–5years,the second for 5–10years and the third one included those aged more than 10 years. All patients met diagnostic guidelines established by Egyptian Ministry of health. Results: out of the positive 22 (25%) patients, 13(59.1%) of them were male, while 9 (40.9%) were females. All enrolled patients have a history of near contact exposure (100%). Thrombocytopenia was the highest presenting symptom in all enrolled patients18(81.8%), while other hematological findings were anemia in 11 (50%), thrombotic symptoms in 2(9.1%), pancytopenia in 2(9.1%) while bleeding was found in 1 patient (4.5%) .Fever 16 (72.7%) the common constitutional symptoms in COVID-19 were not reported in all enrolled patients (0%) while sore throat was reported in only 2 patients (9.1%).Respiratory presentation was only dominant in positive chest CT finding rather than clinical symptoms 17(72.3%) GIT symptom were the dominant presenting feature as vomiting was found in 15 (68.2%), diarrhea in 10 (45.5%), abdominal pain in 11 (50%), jaundice in 9 (40.9%) and dehydration in 6 (27.3%).Neurological symptoms were convulsions in 4(18.2%) while encephalopathy was 2(9.1%).Nephritis was the only renal presentation in the enrolled patients3 (13.6%).Cardiac presentations were only cyanosis 8 (36.4%) and arrhythmias 6 (27.3%) Conclusion: COVID-19 has many clinical  classic presentation in children  however  other non-typical presentation like hematological. CNS and renal presentation has been reported.   
      PubDate: Sat, 01 Jan 2022 00:00:00 +000

    • Authors: Moussa Seck; Alioune Badara Senghor, Mossane Loum, Sokhna Aissatou Touré, Blaise Félix Faye, Alioune Badara Diallo, Mohamed Keita, Elimane Seydi Bousso, Sérigne Mourtalla Guèye, Macoura Gadji, Abibatou Sall, Awa Oumar Touré, Saliou Diop
      Abstract: Context and Objectives: Blood transfusions (BT) remain a mainstay of therapy for patients with sickle cell disease (SCD), but pose significant clinical challenges. We aim to assess infectious markers, red cell alloimmunization and iron overload secondary to BT in SCD patients. Materials and Methods: This is a case-control study included 253 SCD (153 SCD-transfused and 100 SCD non-transfused). We evaluated the transfusion practice (modalities, indications), post-transfusion complications (infections, alloimmunization, iron overload) and risk factors of these complications (socio-demographic, clinical, biological). Results: Median age was 28.5 years (5 - 59). Sex ratio was 0.86. Homozygous SCD was more common (95.3%). Simple BT was performed in 92.8% and transfusion exchange in 18.9%. Transfusion indications were dominated by acute anemia (57.06%) and vaso-occlusive crisis (VOCs) (14%). Red blood cell concentrates (RBC) were administered to 93.46%. Median number of RBC received per patient was 10 (2 - 48). The prevalence of VHC in SCD-transfused was 1.33% and 2% for VHB. Anti-HIV antibodies were not found. Red cell alloimmunization frequency was 16%. The most common alloantibodies were anti-rhesus (34.19%) and anti-Kell (23.67%). Iron overload was detected in 7.84%. The number of RBC transfused was the only risk factor for alloimmunization (p = 0.03) and iron overload (p = 0.023). BT frequency was not related to infectious transmission. Conclusion: Despite advances in blood safety, BT therapy is still a risk for SCD polytransfused patients. Although infectious transmission has rare, the risk of alloimmunization and iron overload is high in these patients.
      PubDate: Sat, 01 Jan 2022 00:00:00 +000

    • Authors: Hong Li; Kehong Bi, Saran Feng, Yan Yan Wang, Chuansheng Zhu
      Abstract: Objectives:MiR-140 and DNAJC3-AS1 have been proven to play critical roles in cancer biology, while their participations in acute myeloid leukemia (AML) are unclear. This study aimed to explore the role of miR-140 and DNAJC3-AS1 in AML. Methods:Analysis of the expression of DNAJC3-AS1 and miR-140 was done with RT-qPCR. The role of DNAJC3-AS1 and miR-140 in the expression of each other was explored with overexpression assay. The direct interaction between DNAJC3-AS1 and miR-140 was analyzed with RNA pull-down assay. The subcellular location of DNAJC3-AS1 was explored with cellular subcellular fractionation assay. Cell proliferation analysis was done with BrdU assay. Results:AML patients showed increased expression of DNAJC3-AS1 and decreased expression of miR-140. DNAJC3-AS1 was detected in both nuclear and cytoplasm samples, and a direct interaction between DNAJC3-AS1 and miR-140 was observed.   Discussion:Reduced expression of DNAJC3-AS1 was observed after miR-140 overexpression in AML cells. DNAJC3-AS1 increased cell proliferation and inhibited the role of miR-140 in suppressing cell proliferation. Conclusion:In conclusion, miR-140 may target DNAJC3-AS1 to suppress cell proliferation in AML.
      PubDate: Sat, 01 Jan 2022 00:00:00 +000

    • Authors: Stefano Botti; Chiara Cannici, Sarah Liptrott, Valentina De Cecco, Elena Rostagno, Gianpaolo Gargiulo, Laura Orlando , Caime Alessandro, Emanuela Samarani, Letizia Galgano, Marco Cioce, Nicola Mordini, Nadia Mandelli, Lucia Tombari, Sara Errichiello, Nicola Celon, Roberto Lupo; Teresa Rea; Nicola Serra
      Abstract: Background and objective: Northern Italy was one of the first European territories to deal with the Coronavirus Disease 2019 (COVID-19) outbreak. Drastic emergency restrictions were introduced across the country to contain the spread and limit pressure on healthcare facilities. Nurses were at high risk of developing physical, mental and working issues due to professional exposure. The aim of this cross-sectional study was to investigate these issues among nurses working in Italian hematopoietic stem cell transplant (HSCT) programmes during the COVID-19 pandemic. Methods: Data were collected online immediately after the first "lockdown" period in order to investigate the prevalence of physical issues, sleep disorders and burnout symptoms and explore correlations with COVID-19 territorial incidence in Northern Italian regions versus Central and Southern Italian regions. Results: Three hundred and eight nurses working in 61 Italian HSCT Units responded to the survey. Depression, cough and fever were more frequently reported by nurses working in geographical areas less affected by the pandemic (p=0.0013, p<0.0001 and p=0.0005 respectively) as well as worst sleep quality (p=0.008). Moderate levels of emotional exhaustion (mean±SD - 17.4±13.0), depersonalization (5.3±6.1) and personal accomplishment (33.2±10.7) were reported without significant differences between territories. Conclusions: different COVID-19 incidence among territories did not influenced nurses’ burden of symptoms in HSCT setting. However, burnout and insomnia levels should be considered by health care facilities in order to improve preventive strategies.
      PubDate: Sat, 01 Jan 2022 00:00:00 +000
  • Coexistence of T-Large Granular Lymphocyte Leukemia and Peripheral T Cell
           Lymphoma-NOS with indolent behaviour

    • Authors: Luca Guarnera; Valentina Boldrini, Gianmario Pasqualone, Carolina Cimino, Elisa Meddi, Roberta Laureana, Donata Trivigno, Giovanni Del Poeta, Alessandro Mauriello, Lucia Anemona, Massimiliano Postorino, Maria Cantonetti
      Abstract: T-cell lymphomas and leukemias are highly heterogeneous groups of rare disorders. We report a case of a 68-year-old man patient who develops two different T-cell neoplasms (Large Granular Lymphocyte Leukemia [LGLL] in 2018 and Peripheral T-cell non-Hodgkin lymphoma  not otherwise specified [PTCL-NOS] in 2019) with a previous diagnosis of B-cell marginal zone lymphoma in 2010, treated with two lines of chemo-immunotherapy. The coexistence of these different T-cell neoplasms is rarely reported in literature and, moreover, is usually described as an LGLL transformation into PTCL-NOS; differently from these examples, herein the simultaneous conditions appear to be driven by different T-cell clones. Furthermore, the PTCL-NOS had a quite unusual behaviour, with a good disease control without intensive treatment. Because of these features, it could belong to a subgroup of indolent PTCL-NOS, not yet described in the WHO classification of T-cell neoplasms, which could benefit of less aggressive treatment.
      PubDate: Sat, 01 Jan 2022 00:00:00 +000
  • Obituary of Prof. Eligio Pizzigallo

    • Authors: Giuseppe Leone
      Abstract: Obituary fo Prof. Eligio Pizzigallo
      PubDate: Sat, 01 Jan 2022 00:00:00 +000
  • Obituary of Prof. Michele Baccarani

    • Authors: Elisabetta Abruzzese
      Abstract: Obituary of Prof. Baccarany
      PubDate: Sat, 01 Jan 2022 00:00:00 +000
  • VEGF and IL-6 correlation in POEMS: a potential upcoming marker of active
           disease and early autologous BMT response.

    • Authors: Luca Laurenti; Idanna Innocenti, Giulia Benintende, Annamaria Tomasso, Francesco Autore, Alberto Fresa, Florenzia Vuono, Silvia Baroni, Claudia Giannotta, Patrizia Chiusolo, Federica Sorà, Simona Sica
      Abstract: Introduction: VEGF function may be responsible for most POEMS manifestations, and it is considered a reliable marker of disease. COVID-19 era arose increasing interest for other inflammatory cytokines, with particular focus on Interleukin-6; VEGF production is stimulated by IL-6 and IL1β, whose concentrations appear to be elevated in clonal plasma cells. Objectives: This study aims to simultaneously evaluate VEGF and IL-6 values in patients (pts) with POEMS at different stages of the disease to find a correlation between them. Methods: We performed a monocentric study, measuring serum levels of VEGF and IL-6 in 8 POEMS pts at different time points of the disease. Results: We observed elevated serum levels of both VEGF and IL-6 in three pts before transplant, while the day after the infusion of autologous stem cells, we observed a steep decrease of both serum markers. Among the four-pts tested only after transplant, two presented with a consensual level of VEGF and IL-6, while the others did not correlate. One patient observed at POEMS diagnosis, during active disease, presented with strikingly high levels of both serum markers. Conclusions: So far, to the best of our knowledge, IL-6 could be considered as a marker of active disease and reliable up to the very first months after BMT, after which its accuracy appears to be lost due to unknown factors, still to be investigated.
      PubDate: Sat, 01 Jan 2022 00:00:00 +000
  • Severe immune thrombocytopenia (ITP) following SARS-CoV-2 mRNA vaccine in
           a girl on immunosuppressive treatment and in prolonged stable phase of

      Abstract: Rare cases of immune thrombocytopenia (ITP) occurring after SARS-CoV-2 mRNA vaccines have recently reached public attention. It has been reported in patients with previous ITP or other autoimmune diseases and in individuals with an apparent negative past medical history.  The management and the outcome of these cases are still not well investigated and reported in the medical literature. A 23-year-old female with a past medical history of ITP, in stable complete remission for 3 years and on mycophenolate treatment received SARS-CoV-2 mRNA vaccine. She presented severe ITP recurrence with hemorrhagic symptoms after the second vaccine dose. A combined treatment with high-dose immunoglobulin and prednisone was successfully administered with a full recovery of platelet count. The patient remains in ITP remission and on mycophenolate therapy, five months later. At our Center, none of the other 76 adult “fragile patients” with ITP on immunosuppressive treatment who had received the SARS-CoV-2 mRNA vaccine, developed such a severe thrombocytopenic recurrence. Follow-up of large cohorts of patients receiving mRNA vaccine will answer the question as to whether it increases the risk of autoimmune conditions. So far, the benefits of the vaccination largely outweigh the risk of infection in these patients.
      PubDate: Sat, 01 Jan 2022 00:00:00 +000
  • Clinical and Prognostic Features in a Young Adult Patient with de novo
           Myelodysplastic Syndrome presenting t(11;16) (q23; q24)

    • Authors: Viviane Lamim Lovatel; Luize Otero, Ercole Pietro Orlando, Claudia Diniz, Filipe Vicente dos Santos-Bueno, Bruno Almeida Lopes, Elaiza Almeida Antônio de Kós, Monica Kopischitz Praxedes Lusis, Eliana Abdelhay, Teresa de Souza Fernandez
      Abstract: hematopoietic clonal neoplasms. MDS occurs mainly in elderly patients. KMT2A rearrangements (KMT2A-r) are rare in MDS, so little is known about their prognostic value. The present study describes the clinical characteristics of a young adult patient diagnosed with MDS-EB-2, presenting the t(11;16)(q23;q24). The Decitabine treatment was initiated since no matching donor was found. The patient showed improved anemia and thrombocytopenia. However, he still had severe neutropenia and clonal chromosomal alteration.   Two months after the fifth cycle of Decitabine, the patient presented a worsening of the clinical parameters with increased blast and evolution to AML. He was treated with intensification chemotherapy, but despite all efforts, the patient evolved to death. Treatment refractoriness and leukemia transformation suggest that t(11;16)(q23;q24) with KMT2A-r was associated with poor prognosis. This study reinforces the importance of characterizing new chromosomal alterations and their impact on prognosis in MDS.
      PubDate: Sat, 01 Jan 2022 00:00:00 +000
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Heriot-Watt University
Edinburgh, EH14 4AS, UK
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