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Mediterranean Journal of Hematology and Infectious Diseases
Number of Followers: 0  Open Access journalISSN (Print) 2035-3006 This journal is no longer being updated because: the publisher no longer provides RSS feeds |
- LYMPHOCYTES IN PATIENTS WITH CHRONIC ACTIVE EPSTEIN-BARR VIRUS INFECTION
EXHIBITED ELEVATED PD-1/PD-L1 EXPRESSION AND A PREVAILING TH2 IMMUNE
RESPONSE.
Authors: kang sun
Abstract: Background And Objectives: Chronic active Epstein-Barr virus infection (CAEBV) is a lymphoproliferative disorder characterized by the increased numbers of EBV-infected T/natural killer (NK) cells and ongoing symptoms resembling infectious mononucleosis. Nonetheless, the exact correlation between peripheral lymphocyte subsets and the development of CAEBV remains uncertain. Methods: Consequently, we examined the levels of programmed cell death 1 (PD-1)/programmed death ligand 1 (PD-L1) expression, the status of EBV infection, and the frequencies of peripheral lymphocyte subpopulations in 24 patients with CAEBV and 15 EBV-seronegative donors using flow cytometry. Results: Patients with CAEBV showed a notable rise in the expression levels of PD-1 and PD-L1 in peripheral T and NK cells compared to healthy donors (P < 0.05). The induction of PD-L1 expression was attributed to EBV infection. Moreover, most of the T cells infected with EBV displayed a memory phenotype characterized by the presence of CD45RO+. Additionally, patients with CAEBV showed markedly decreased frequency of helper T cells 1 (Th1) and naïve T (Tn) cells, and significantly elevated frequency of Th2 and effector-memory T (Tem) cells. Conclusions: To summarize, EBV might evade the immune system by inducing PD-1/PD-L1 expression in peripheral T and NK cells, and promoting Th2 immune response dominance. Additionally, it may establish long-term persistence by infecting memory T cells in CAEBV.
PubDate: Tue, 30 Apr 2024 00:00:00 +000
- CLINICAL AND LABORATORY FEATURES OF SICKLE CELL DISEASE S/D PUNJAB: IMPACT
OF HBF AND HYDROXYUREA
Authors: r. Salam Al-Kindi; Isra Al-Busaidi, Anil Pathare
Abstract: Background: Sickle cell disease (SCD) is a major public health issue worldwide with high morbidity and mortality. SCD SD Punjab is the third most common genotype of SCD in Oman and is associated with several serious complications. The aim of the study is to establish the clinical and laboratory features of SCD patients with SD double heterozygotes and study the impact of haemoglobin F, hydroxyurea, and other modulators on the disease severity. Methods: We analyzed the electronic medical records of 52 consecutive SCD patients who were diagnosed as double heterozygote SD Punjab between 2006 and 2022. The study was approved by the local medical research and ethics committee. The data captured included SCD-related complications and current clinical and laboratory indices. Data from other studies on other SCD genotypes were used as historical controls. Results: 52 patients (31 males, 21 females) who formed this cohort had a median age of 32 years with an interquartile range (IQR) of 21-39.8 years. 37(71.2%) had <3 VOC per year, whereas 15 (28.8%) patients had >3 vasooclusive (VOC) episodes per year. SCD-related complications included Acute Chest Syndrome (ACS) (48%), Gall stones (26.9%), Avascular necrosis (AVN) (28.8%), Stroke (13.5%) and splenic sequestration (7.7%), whereas 5 (9.6%) patients of this cohort died. Surgical and Autosplenectomy were seen in 18 (34.6%). These findings were similar to other SCD genotypes in this community.19 (57.6%) were taking Hydroxyurea (HU) amongst the 33 patients who were prescribed HU. Haematological parameters showed a median (IQR) Hb (g/dl), MCV (fl), Retic count (%), WBC count(X109/L) and Platelet count(X109/L) of 9.7 (8.5-11.3), 74.9 (68.4-79.8), 4 (3.2-5.7), 9.9 (8.1-12.6) and 309 (239-428) respectively. The haemoglobin electrophoresis showed an elevated HbF, whereas serum bilirubin and LDH were elevated amongst the biochemical parameters. The use of hydroxyurea showed no impact on VOC, ACS, AVN, Stroke or mortality. Conclusion: SD Punjab is the third most common SCD genotype in Oman and was associated with recurrent VOC, ACS, AVN, and gall stones comparable to other SCD genotypes. Patients with > 3 VOC/year had significantly increased incidence of Stroke, AVN, and gallstones. However, HU was not associated with improved prognosis and better survival in this cohort of patients. Keywords: Sickle Cell Disease; Haemoglobin SD Punjab; vaso-occlusive crises; acute chest syndrome; Avascular Necrosis; HbF; hydroxyurea.
PubDate: Tue, 30 Apr 2024 00:00:00 +000
- LETERMOVIR PRIMARY CYTOMEGALOVIRUS PROPHYLAXIS IN ALLOGENEIC HEMATOPOIETIC
CELL TRANSPLANT RECIPIENTS: COULD INFECTION AND DISEASE NO LONGER BE A
SIGNIFICANT PROBLEM'
Authors: Fabian Herrera
Abstract: Background: Cytomegalovirus (CMV) infection remains the most common clinically significant infection after allogeneic hematopoietic stem cell transplantation (allo-HCT) and is associated with considerable morbidity and mortality. Objectives: The present study was designed to describe and compare the incidence of untreated CMV reactivation (uCMVr), clinically significant infection (cs-CMVi) and disease (CMVd), as well as CMV-related hospitalization and outcome of allo-HCT patients, either treated with letermovir (LET) primary prophylaxis or managed with preemptive therapy (PET). Methods: This is a prospective observational cohort study of adult CMV seropositive allo-HCT patients who either received primary prophylaxis with LET within the first 100 days after HCT or were managed with PET. Results: The study population comprised 105 patients (28 in the LET group and 77 in the PET group). Compared to the PET group, patients in the LET group received more allo-HCT from alternative donors (54.5% vs. 82.14%, P=0.012). More than half of the patients in both groups were classified as high risk for CMVd. In the LET vs. PET group, cs-CMVi and CMVd developed respectively in 0 vs. 50 (64.94%), P=<0.0001, and 0 vs. 6 (7.79%), P=0.18. In the LET group, uCMVr occurred in 5 (17.8%) and were all considered blips. Hospital admissions related to cs-CMVi or CMVd in the PET group vs. LET group were 47 (61.04%) vs. 0, respectively, P=<0.0001. No differences were observed in 100-day mortality. Conclusions: LET primary prophylaxis proved effective in preventing cs-CMVi and CMVd and reducing hospitalizations in allo-HCT adults. Blips can occur during prophylaxis and do not require LET discontinuation. Keywords: letermovir; cytomegalovirus; hematopoietic cell transplantation.
PubDate: Tue, 30 Apr 2024 00:00:00 +000
- HAPLOIDENTICAL HEMATOPOIETIC STEM CELL TRANSPLANTATION FOR PAEDIATRIC
PATIENTS WITH X-LINKED LYMPHOPROLIFERATIVE SYNDROME
Authors: Yuan Sun
Abstract: The aim of this study was to investigate the prognostic factors of haploid hematopoietic stem cell transplantation in the treatment of X-linked lymphoproliferative syndrome. Seven children with X-linked lymphoproliferative syndrome diagnosed by XIAP gene analysis were enrolled. The conditioning regimens were tolerated in all seven patients, and the median time of neutrophil engraftment was 10 days (8-13 days), that of platelet engraftment was 21 days (14-24 days). STR-PCR analysis on the peripheral blood cells showed complete donor origins. Four cases developed Grade I acute graft versus host disease (aGVHD), one developed Grade III aGVHD (intestinal tract), and two cases had limited chronic GVHD. Four cases had cytomegalovirus (CMV) reactivation, and two cases had Epstein–Barr virus (EBV) reactivation. One case was diagnosed as pneumocystosis, and thrombotic microangiopathy (TMA) occurred in three cases. During the follow-up period (median time of 42 months), one patient died of TMA and six patients survived. Statistical analysis showed that the status of disease remission and the positive result of virus in blood before transplantation were independent prognostic factors. Haplo-HSCT might be a curative option for children with refractory X-linked lymphoproliferative syndrome. Low-intensity conditioning regimens may reduce transplant-related mortality and improve overall survival.
PubDate: Tue, 30 Apr 2024 00:00:00 +000
- Impact of the Addition of Daratumumab to the Standard
Bortezomib-Thalidomide-Dexamethasone Regimen on Hematopoietic Stem Cell
Mobilization and Collection, Post-Transplant Engraftment and Infectious
Complications: A Case-Control Multicentre Real-Life Analy
Authors: Gianluca Cavallaro
PubDate: Tue, 30 Apr 2024 00:00:00 +000
- Antivirals and monoclonal antibody combination therapy in haematological
patients in the omicron era
Authors: Serena Vita; Emanuela Giacobini, Patrizia De Marco, Martina Rueca, Cesare Ernesto Maria Gruber, Alessia Beccacece, Laura Scorzolini, Valentina Mazzotta, Carmen Pinnetti, Priscilla Caputi, Daniele Focosi, Enrico Girardi, Andrea Antinori, Fabrizio Maggi, Alessandra D'Abramo, Emanuele Nicastri, Spallanzani COVID-19 case Investigation Team
Abstract: Abstract: Im We describe here a single-center case series of 27 IC COVID-19 inpatients (mostly with haematological disorders) treated with a combined therapy based on tixagevimab/cilgavimab (T/C) plus small-molecule antivirals (AV), between April 1 2022 and November 30 2022. Keywords: immunocompromised; SARS-CoV-2 infection; monoclonal antibodies; antivirals; persistent infection; viral evolution
PubDate: Tue, 30 Apr 2024 00:00:00 +000
- Impact of daratumumab on stem cell mobilization and transplant in patient
with newly diagnosed multiple myeloma: a real word single-center study
Authors: Mauro Passucci; Francesca Fazio, Jacopo Micozzi, Manhaz Shafii Bafti, Giovanni Manfredi Assanto, Alfonso Piciocchi, Maurizio Martelli, Maria Teresa Petrucci
Abstract: Not applicable.
PubDate: Tue, 30 Apr 2024 00:00:00 +000
- CAR-T CELLS IN CHRONIC LYMPHOCYTIC LEUKEMIA
Authors: Ugo Testa; Dr. Elvira Pelosi, Dr. Germana Castelli, Dr. Alberto Fresa, Prof. Luca Laurenti
Abstract: The treatment outcomes of patients with chronic lymphocytic leukemia (CLL) have considerably improved with the introduction of targeted therapies based on Bruton kinase inhibitors (BTKIs), venetoclax, and anti-CD20 monoclonal antibodies. However, despite these consistent improvements, patients who become resistant to these agents have poor outcomes and need new and more productive therapeutic strategies. Among these new treatments, a potentially curative approach consists of the use of chimeric antigen receptor T (CAR-T) cell therapy, which achieved remarkable success in various B-cell malignancies, including B-cell Non-Hodgkin Lymphomas (NHLs) and B-acute lymphoblastic Leukemia (ALL). However, although CAR-T cells were initially used for the treatment of CLL, their efficacy in CLL patients was lower than in other B-cell malignancies. This review analyses the possible mechanisms of these failures. It highlights some recent developments that could offer the perspective of the incorporation of CAR-T cells in treatment protocols for relapsed/refractory CLL patients. Keywords: Chronic Lymphoid Leukemia; CLL; CAR-T Cells; Relapsed/resistant CLL:
PubDate: Tue, 30 Apr 2024 00:00:00 +000
- IS IT POSSIBLE TO PREDICT TUMOR PROGRESSION THROUGH GENOMIC
CHARACTERIZATION OF MONOCLONAL GAMMOPATHY AND SMOLDERING MULTIPLE MYELOMA'
Authors: Ugo Testa; Prof. Leone, Dr. Elvira Pelosi, Dr. Germana Castelli, Prof, Valerio De Stefano
Abstract: The study of monoclonal serum proteins has led to the generation of two major theories: one proposing that individuals who had monoclonal proteins without any symptoms or evidence of end-organ damage have a benign condition; the other one suggesting that some individuals with asymptomatic monoclonal proteins may progress to multiple myeloma and thus are affected by a monoclonal gammopathy of undetermined significance (MGUS). Longitudinal studies of subjects with MGUS have supported the second theory. Subsequent studies have characterized and defined the existence of another precursor of multiple myeloma, smoldering multiple myeloma (SMM), intermediate between MGUS and multiple myeloma. Primary molecular events, chromosome translocations, and chromosome number alterations resulting in hyperploidy, required for multiple myeloma development, are already observed in myeloma precursors. MGUS and SMM are heterogeneous conditions with the presence of tumors with distinct pathogenic phenotypes and clinical outcomes. The identification of MGUS and SMM patients with a molecularly defined high risk of progression to MM offers the unique opportunity of early intervention with a therapeutic approach on a low tumor burden.
PubDate: Tue, 30 Apr 2024 00:00:00 +000
- CLINICAL CARE PATHWAY AND MANAGEMENT OF MAJOR BLEEDING ASSOCIATED WITH
NON-VITAMIN K ANTAGONIST ORAL ANTICOAGULANTS: A MODIFIED DELPHI CONSENSUS
FROM SAUDI ARABIA AND UAE
Authors: Abdulrahman Al Raizah; Fakhr Alayoubi, Galal Hassan Abdelnaby, Hazzaa Alzahrani, Majid Farraj Bakheet, Mohammed A Alskaini, Rasha Buhumaid, Sameer Al Awadhi, Sara Nooruddin Kazim, Thiagarajan Jaiganesh, Zohair Al Asiri
Abstract: Background: The non-vitamin K antagonist oral anticoagulants (NOACs) have become the mainstay anticoagulation therapy for patients requiring oral anticoagulants (OACs) in the Gulf Council Cooperation (GCC) countries. The frequency of NOAC-associated major bleeding is expected to increase in the Emergency Department (ED). Nonetheless, we still lack local guidelines and recommendations for bleeding management in the region. The present Delphi-based consensus aims to establish a standardized and evidence-based clinical care pathway for managing NOAC-associated major bleeding in the Kingdom of Saudi Arabia (KSA) and the United Arab Emirates (UAE). Methods: We adopted a three-step modified Delphi method to develop evidence-based recommendations through two voting rounds and an advisory meeting between the two rounds. A panel of 11 experts from the KSA and UAE participated in the consensus development. Results: Twenty-eight statements reached the consensus level. These statements addressed key aspects of managing major bleeding events associated with NOACs, including the increased use of NOAC in clinical practice, clinical care pathways, and treatment options. Conclusion: The present Delphi consensus provides evidence-based recommendations and protocols for the management of NOAC-associated bleeding in the region. Patients with major NOAC-induced bleeding should be referred to a well-equipped ED with standardized management protocols. A multidisciplinary approach is recommended for establishing the association between NOAC use and major bleeding. Treating physicians should have prompt access to specific reversal agents to optimize patient outcomes. Real-world evidence and national guidelines are needed to aid all stakeholders involved in NOAC-induced bleeding management.
PubDate: Tue, 30 Apr 2024 00:00:00 +000
- EBV-RELATED LYMPHOPROLIFERATIVE DISEASES: A REVIEW IN LIGHT OF NEW
CLASSIFICATIONS
Authors: Pietro Tralongo; Arianna Bakacs, Luigi Maria Larocca
Abstract: Epstein-Barr virus (EBV) is a prevalent virus that can be detected in the vast majority of the population. Most people are asymptomatic and remain chronically infected throughout their lifetimes. However, in some populations, EBV has been linked to a variety of B-cell lymphoproliferative disorders (LPDs), such as Burkitt lymphoma, classic Hodgkin lymphoma, and other LPDs. T-cell LPDs have been linked to EBV in part of peripheral T-cell lymphomas, angioimmunoblastic T-cell lymphomas, extranodal nasal natural killer/T-cell lymphomas, and other uncommon histotypes. This article summarizes the current evidence for EBV-associated LPDs in the light of the upcoming World Health Organization classification and the 2022 ICC classification. Keywords: Epstein Barr Virus: EBV, Lymphoma; Lymphoproliferative Diseases.WHO Classification.
PubDate: Tue, 30 Apr 2024 00:00:00 +000
- Outcome of Allogeneic Hematopoietic Stem Cell Transplantation in a Child
with Myelodysplastic Neoplasm with Complex Karyotype and ETV6 Variant:
Challenges in Treatment
Authors: Elaiza Almeida Antônio de Kós ; Viviane Lamim Lovatel , Rita de Cássia Barbosa Tavares , Gerson Moura Ferreira , Bernadete Evangelho Gomes, Ana Paula Silva Bueno, Elaine Sobral da Costa , Teresa de Souza Fernandez
Abstract: Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is the only treatment with the potential for cure in patients with myelodysplastic neoplasm (MDS). Nevertheless, disease relapse is the main cause of treatment failure. Due to the rarity of childhood MDS (cMDS), there are few studies showing the impact of cytogenetic alterations and genetic variants on outcomes of allo-HSCT, mainly focusing on clonal chromosomal evolution (CCE) during relapse post-transplant. Here, we describe a 3-year-old boy with cMDS-IB, who evolved to MDS/AML.
PubDate: Tue, 30 Apr 2024 00:00:00 +000
- Very late onset Post-Transplantation Lymphoproliferative Disorder (PTLD)
after haematopoietic stem cell transplant (HCT) – A Clinical Case
Authors: Sharon Lionel; Liron Barnea Slonim, Guy Hannah, Victoria Potter, Daniele Avenoso
PubDate: Tue, 30 Apr 2024 00:00:00 +000
- Evaluation of VTE-PREDICT risk score in patients receiving low-dose DOACs
for venous thromboembolism (VTE) secondary prophylaxis
Authors: Alessandro Laganà; Giovanni Manfredi Assanto, Mauro Passucci, Cristina Santoro, Antonio Chistolini
PubDate: Tue, 30 Apr 2024 00:00:00 +000
- The THE PLASMABLASTIC LYMPHOMA. A STATE-OF-THE-ART REVIEW: PART 2-FOCUS ON
THERAPY
Authors: Michele Bibas
Abstract: The objective of this two-part review is to present a current and comprehensive understanding of the diagnosis and management of plasmablastic lymphoma. The first part, which was published previously, focused on the study of epidemiology, etiology, clinopathological characteristics, differential diagnosis, prognostic variables, and the impact of plasmablastic lymphoma on specific populations. This second part addresses the difficult topic of the treatment of plasmablastic lymphoma, specifically examining both the conventional, consolidated approach and the novel therapeutic strategy.
PubDate: Thu, 29 Feb 2024 00:00:00 +000
- HOW THE HEMOSTASIS LABORATORY CAN HELP CLINICIANS TO MANAGE PATIENTS ON
ORAL ANTICOAGULANTS
Authors: Armando Tripodi
Abstract: Oral anticoagulants are widely used to treat or prevent cardiovascular diseases in millions of patients worldwide. They are the drugs of choice for stroke prevention and systemic embolism in patients with non-valvular atrial fibrillation and prosthetic heart valves, as well as for treatment/prevention of venous thromboembolism. Oral anticoagulants include the vitamin K antagonists (VKAs) and direct oral anticoagulants (DOACs). The hemostasis laboratory plays a crucial role for the management of treated patients that spans from dose-adjustment based on laboratory testing that applies to VKAs to the measurement of drug concentrations in special situations that applies to DOACs. This article aims to overview how the hemostasis laboratory can help clinicians to manage patients on oral anticoagulants. Special interest is devoted to the international normalized ratio, used to manage patients on VKAs and to the measurement of DOAC concentrations, for which the role of the laboratory is still not very well defined and most interferences of DOACs with some of the most common hemostatic parameters are not widely appreciated.
PubDate: Thu, 29 Feb 2024 00:00:00 +000
- CAR-T CELL THERAPY FOR T-CELL MALIGNANCIES
Authors: Ugo Testa; Prof. Patrizia Chiusolo, Dr. Elvira Pelosi, Dr. Germana Castelli, Giuseppe Leone
Abstract: Chimeric antigen receptor T-cell (CAR-T) therapy has revolutionized the treatment of B-cell lymphoid neoplasia and, in some instances, improved disease outcomes. Thus, six FDA-approved commercial CAR-T cell products that target antigens preferentially expressed on malignant B-cells or plasma cells have been introduced in the therapy of B-cell lymphomas, B-ALLs and multiple myeloma. These therapeutic successes have triggered the application of CAR-T cell therapy to other hematologic tumors, including T-cell malignancies. However, the success of CAR-T cell therapies in T-cell neoplasms was considerably more limited to the existence of some limiting factors, such as the sharing of mutual antigens between normal T-cells and CAR-T cells, and malignant cells, determining fratricide events and severe T-cell aplasia; contamination of CAR-T cells used for CAR transduction with contaminating malignant T-cells. Allogeneic CAR-T products can avoid tumor contamination but raise other problems related to immunological incompatibility. In spite of these limitations, there has been significant progress in CD7- and CD5-targeted CAR-T cell therapy of T-cell malignancies in the last few years.
PubDate: Thu, 29 Feb 2024 00:00:00 +000
- COMPARISON OF INFECTIOUS COMPLICATIONS IN PATIENTS RECEIVING HIGH-DOSE
CYCLOPHOSPHAMIDE AS GVHD AFTER TRANSPLANTATION FROM A 9/10 HLA-MATCHED
UNRELATED DONOR WITH STANDARD GVHD PROPHYLAXIS AFTER TRANSPLANT FROM A
FULL MATCHING DONOR
Authors: Selim SAYIN
Abstract: Background: The aim of this study was to evaluate whether cyclophosphamide administered after allogeneic stem cell transplantation (ASCT) from 9/10 HLA-Matched Unrelated Donors (MMUD) increases the rates of bloodstream infections (BSI) (fungal, viral (CMV, BK, hepatitis), bacterial), infectious complications (hemorrhagic cystitis(HC)) and infection-related mortality compared to allogeneic stem cell transplantation from matched related donors (MRD). Metods: This is a retrospective multicenter study. 45 MMUD ASCT patients who received posttransplant cyclophosphamide + methotrexate + calcineurin inhibitor compared with 45 MRD ASCT patients who received methotrexate + calcineurin inhibitor. Results: Although there was a statistically significant prolongation of neutrophil engraftment time in the PTCy arm, there was no statistically significant difference in bacterial BSI frequencies between the groups (PTCy; 9(20%), control;8 (17.8%), p=0.778). The distribution of CMV infection in the first 100 days was similar (p=0.827) but the distribution of CMV infection rate between the 100th and 365th days, was observed more frequently in the control group (p=0.005). HC rates and their grades were similar in both groups (PTCy; 4 (8.8%), control;6 (13.3%) p=0.502). The rates of VZV infection and invasive aspergillosis were similar in the PTCy and control groups (13.3% in the PTCy, and 17.8% in the control group p=0.561). IRM rate was statistically similar in both groups (13.3% in the PTCy arm and 17.8% in the control arm) Conclusions: The addition of PTCy to standard GvHD prophylaxis in MMUD ASCT does not lead to an increase in CMV reactivation, bacterial BSI, invasive fungal infection, viral hemorrhagic cystitis or infection-related mortality.
PubDate: Thu, 29 Feb 2024 00:00:00 +000
- NG2 MOLECULE EXPRESSION IN ACUTE LYMPHOBLASTIC LEUKEMIA B CELLS: A
FLOW-CYTOMETRIC MARKER FOR THE RAPID IDENTIFICATION OF KMT2A GENE
REARRANGEMENTS
Authors: Maria Laura Bisegna; Nadia Peragine, Loredana Elia, Mabel Matarazzo, Maria Laura Milani, Stefania Intoppa, Mariangela Di Trani, Francesco Malfona, Maurizio Martelli, Maria Stefania De Propris
Abstract: Background: B-lineage acute lymphoblastic leukemias (B-ALL) harboring rearrangements of the histone lysine [K]-Methyltransferase 2A (KMT2A) gene on chromosome 11q23 (KMT2A-r), represent a category with dismal prognosis. The prompt identification of these cases represents an urgent clinical need. Considering the correlation between rat neuron glial-antigen 2 (NG2) chondroitin sulfate proteoglycan molecule expression and KMT2A-r, we aimed identifying an optimized cytofluorimetric diagnostic panel to predict the presence of KMT2A-r. Materials and Methods: We evaluated 88 NG2+ B-ALL cases identified with a NG2 positivity threshold >10%, from a cohort of 1382 newly diagnosed B-ALLs referred to the Division of Hematology of ‘Sapienza’ University of Rome. Results: Eighty-five of 88 (96.6%) NG2+ B-ALLs harbored KMT2A-r and were mainly pro-B ALL (77/85; 91%). Only 2 B-ALLs with KMT2A-r showed NG2 expression below 10%, probably due to the steroid therapy administered prior to cytofluorimetric analysis. When compared to KMT2A-r‒ cases, KMT2A r+ B-ALLs presented a higher blast percentage, a significantly higher mean fluorescence intensity (MFI) of CD45, CD38 and CD58, and a significantly lower CD34, CD22, TdT and CD123 MFI. The differences in CD45, CD34, CD22 and TdT MFI were confirmed within the same immunologic EGIL (European Group for the immunological classification of leukemias) group, suggesting no influence of the B-ALLs EGIL subtype on the peculiar KMT2A-r+ B-ALLs immunophenotype. Conclusions: Our data show how the association between NG2 and KMT2A-r in B-ALLs identify a peculiar immunophenotypic pattern, useful for rapid identification in diagnostic routines of these subtypes of B-ALLs with a poor prognosis, that benefit from a specific therapeutic approach.
PubDate: Thu, 29 Feb 2024 00:00:00 +000
- RITUXIMAB VERSUS SPLENECTOMY IN CHRONIC PRIMARY ITP: EXPERIENCE OF A
SINGLE HEMATOLOGY CLINIC
Authors: Rawanda Shamoon; Ahmed K. Yassin, Sara L. Alnuaimi
Abstract: Objective: Immune thrombocytopenia (ITP) is an acquired immune-mediated disease that lacks an underlying etiology. Steroids are the main first-line treatment of ITP, while the second-line treatment consists primarily of splenectomy and rituximab. This study aimed to assess and compare the response to rituximab and splenectomy. Methods: This retrospective comparative study reviewed ITP patients treated at a single private hematology clinic from 2007 to 2019. Seventy-four ITP patients were recruited, 27 were on rituximab, and 47 had undergone splenectomy. The initial platelet counts and bleeding symptoms were recorded, and initial and long-term responses to treatment were evaluated based on the American Society of Hematology guidelines. Results: The mean age of the patients was 42.1 years with a male-to-female ratio of 1:1.8. The initial mean platelet count was comparable between the rituximab and splenectomy groups (p = 0.749). The initial complete response (CR) differed significantly between the rituximab and splenectomy groups (44.4% versus 83%, p = 0.002). The five-year response rate was significantly higher in the splenectomy than in the rituximab group (74% versus 52%, log-rank 0.038). Splenectomy was the only significant predictive factor for long-term response (OR = 0.193, p = 0.006). Conclusion: The overall response revealed that splenectomy appeared superior to rituximab as a second-line treatment of ITP. Splenectomy was the only positive prognostic indicator of sustained response.
PubDate: Thu, 29 Feb 2024 00:00:00 +000
- SECONDARY PROPHYLAXIS OF VENOUS THROMBOEMBOLISM (VTE) WITH LOW DOSE
APIXABAN OR RIVAROXABAN: RESULTS FROM A PATIENT POPULATION WITH MORE THAN
2 YEARS OF MEDIAN FOLLOW-UP
Authors: Alessandro Laganà; Giovanni Manfredi Assanto, Chiara Masucci, Mauro Passucci, Livia Donzelli, Alessandra Serrao, Erminia Baldacci, Cristina Santoro, Antonio Chistolini
Abstract: Background: Direct oral anticoagulants (DOACs) are widely used for the treatment and secondary prophylaxis of venous thromboembolism (VTE). Nowadays, DOACs represent the gold standard for long-term anticoagulation, with low-intensity DOACs administration becoming increasingly used worldwide in such scenario. Albeit low-intensity apixaban and rivaroxaban are approved for clinical usage as secondary VTE prophylaxis, there are few literature data regarding their efficacy and safety with a long follow-up. Objectives: The aim of our study was to evaluate the efficacy and safety of low-dose DOACs for VTE secondary prophylaxis, in patients at high risk of VTE recurrence. Methods: We retrospectively evaluated patients who required long-term anticoagulant secondary prophylaxis to prevent recurrent VTE, treated with apixaban 2.5 mg BID or rivaroxaban 10 mg daily with a follow-up ≥ 12 months. Results: The examined patients were 323. The median low-dose DOACs administration time was 25.40 months (IQR 13.93-45.90). Twelve (3.7%) VTE recurrences were observed; 21 bleeding events were registered (6.5%), including one episode of MB (0.3%), 8 CRNMB (2.5%) and 12 minor bleeding (3.7%). No statistically significant difference in the rate of VTE recurrence and/or bleeding events emerged between rivaroxaban and apixaban groups. Patients included in the study for multiple episodes of VTE presented a significant higher risk of a new VTE recurrence during low-intensity DOAC. Conclusions: Our data suggest that low-dose DOACs may be effective and safe in the secondary VTE prophylaxis in patients at high risk of VTE recurrence, but attention might be needed in their choice in such scenario for patients who experienced multiple episodes of VTE.
PubDate: Thu, 29 Feb 2024 00:00:00 +000
- COEXISTENCE OF MULTIPLE GENE VARIANTS IN SOME PATIENTS WITH ERYTHROCYTOSES
Authors: Andrea Benetti; Irene Bertozzi, PhD, MD, Giulio Ceolotto, Irene Cortella, Daniela Regazzo, Giacomo Biagetti, Elisabetta Cosi, Maria Luigia Randi, MD, Prof
Abstract: Background: Erythrocytosis is a relatively common condition, however a large proportion of these patients (70%) remain without a clear etiologic explanation. Methods: We set up a targeted NGS panel for patients with erythrocytosis and 118 sporadic patients with idiopathic erythrocytosis were studied. Results: In 40 (34%) patients no variant was found while in 78 (66%) we identified at least one germinal variant; 55 patients (70.5%) had 1 altered gene, 18 (23%) had 2 alterations, and 5 (6.4%) had 3. An altered HFE gene was observed in 51 cases (57.1%), EGLN1 in 18 (22.6%) and EPAS1, EPOR, JAK2, and TFR2 variants in 7.7%, 10.3%, 11.5%, and 14.1% patients, respectively. In 23 patients (19.45%), more than 1 putative variant was found in multiple genes. Conclusions: Genetic variants in patients with erythrocytosis were detected in about 2/3 of our cohort. A NGS panel including more candidate genes should reduce the number of cases diagnosed as “idiopathic” erythrocytosis in whom a cause cannot yet be identified. It is known that HFE variants are common in idiopathic erythrocytosis. TFR2 alterations supports the existence of a relationship between genes involved in iron metabolism and impaired erythropoiesis. Some novel multiple variants were identified. Erythrocytosis appears to be often of multigenic nature.
PubDate: Thu, 29 Feb 2024 00:00:00 +000
- CLINICAL SIGNS AND TREATMENT OF NEW-ONSET BONE MARROW FAILURE ASSOCIATED
SARS-COV-2 INFECTION IN CHILDREN : A SINGLE INSTITUTION PROSPECTIVE COHORT
STUD
Authors: Mervat A M youssef; Ebtisam Shawky hmed, Dalia Tarik Kamal, khalid Elsayh, , Mai A Abdelfatta, Hyam Hassan Mahran Mahran, Mostafa M Embaby
Abstract: Viral infections can cause direct and indirect damage to hematopoietic stem cells. The objectives of this study were to identify the frequency and severity of aplastic anemia in Severe acute respiratory syndrome coronavirus 2 ( SARS-CoV-2) infected children as well as recognition of the response to treatment Methodology: 13 children with newly-diagnosed severe aplastic anemia enrolled in this prospective clinical trial. Blood samples were obtained from all patients to detect SARS-CoV-2 antibodies, and nasopharyngeal swabs were collected for reverse-transcription Polymerase Chain Reaction to detect SARS-CoV-2 viruses. According to the laboratory results, patients were classified in to SARS-CoV-2 positive antibodies and SARS-CoV-2 negative antibodies . Both groups received combined cyclosporine (CsA) + Eltrombopag (E-PAG). The hematological response either complete response (CR)or partial response (PR), no response (NR) and overall response (OR) rates of combined E-PAG + CsA treatment after 6 months were evaluated. Results: Four children were recognized to have aplastic anemia and SARS-CoV-2 positive antibodies. Two patients fulfilled the hematological criteria for CR and no longer required transfusion of packed red blood cells (PRBCs) or platelets and one had PR and were still PRBC transfusion-dependent but no longer required platelet transfusion. The remaining patient showed NR and he had died before reaching the top of the HSCT waiting list. Moreover, six patients in the SARS-CoV-2 negative antibodies group had CR while three patients had PR. The difference in ANC, Hg, and platelet counts between both groups was not significant. Conclusion: SARS-CoV-2 virus is added to several viral infections known to be implicated in the pathogenesis of aplastic anaemia. Studies are needed to establish a definitive association and determine whether the response of bone marrow failure to standard therapy differ from that of idiopathic cases.
PubDate: Thu, 29 Feb 2024 00:00:00 +000
- Antibody response to breakthrough SARS-CoV-2 infection in “booster”
vaccinated patients with multiple myeloma according to B/T/NK lymphocyte
absolute counts and anti-CD38 treatments
Authors: NICOLA SGHERZA; Anna Mestice, Angela Maria Vittoria Larocca, Pellegrino Musto
PubDate: Thu, 29 Feb 2024 00:00:00 +000
- Protein S deficiency with recurrent thromboembolism in a patient with
hemoglobin H disease following splenectomy
Authors: Kun Yang
PubDate: Thu, 29 Feb 2024 00:00:00 +000
- Could the 3′UTR+101G>C Mutation Detected in Two Sibling Cases Be a
Mutation Affecting the Clinical Presentation in Thalassemia Patients'
Authors: Unal Atas; Volkan Karakus, Erdal Kurtoglu
Abstract: Due to letter to aditor, there is no abstract.
PubDate: Thu, 29 Feb 2024 00:00:00 +000
- Clinical Characteristics and Treatment Response of a Novel ELANE Gene
Mutation (c.295_303del) in Congenital Neutropenia
Authors: Junjie Ning
Abstract: NA
PubDate: Thu, 29 Feb 2024 00:00:00 +000
- Successful treatment of de novo acute myeloid leukaemia associated
aortitis by induction chemotherapy alone
Authors: Urbain Tauveron--Jalenques; Vincent Grobost, Benoît Magnin, Cécile Moluçon-Chabrot, Jacques-Olivier Bay, Olivier Tournilhac; Romain Guièze
PubDate: Thu, 29 Feb 2024 00:00:00 +000
- Impact of hydroxyurea on clinical and biological parameters of sickle cell
anemia in children in Abidjan
Authors: MIREILLE YAYO- AYE; Adia Eusèbe Adjambri, Boidy Kouakou, Rebecca N'guessan-Blao, Louis Missa Adjé, Taïratou Kamagaté, Vincent Yapo, Duni Sawadogo
Abstract: Background: The lives of individuals affected by sickle cell disease are marked by painful crises sometimes accompanied by complications. Curative treatments such as bone marrow transplantation or gene therapy exist, but are not currently performed in Côte d'Ivoire. Treatment with hydroxyurea remains an effective alternative. The aim of our study is to contribute to improving the management of children with sickle cell disease. Methods: We conducted a prospective observational study from November 2017 to April 2019 at the at the Yopougon University Hospital. Children aged 5 to 15 years experiencing at least 3 vaso-occlusive crises (VOC) per year were included in the study after obtaining informed and written consent from their parents. Each patient received a daily dose of 15mg/kg of hydroxyurea. Results: The mean age of the children was 9 years. More than 75% of patients were homozygous SSFA2 major sickle cell individuals. After 6 months on hydroxyurea, our study observed rates of 84.4%, 100%, and 97.8%, respectively, for the absence of vaso-occlusive crises, hospitalization, and transfusion. Biologically, from M0 to M12 the mean hemoglobin level increased significantly, from 7.24 to 8.55 g/dL; white blood cell (WBC) and platelet counts decreased; Fetal hemoglobin (Hb F) increased significantly from 10.3% to 19.7%. Biochemical parameters within normal ranges, except for a moderate treatment-related increase in transaminases. Conclusion: The induction of fetal hemoglobin (Hb F) production through hydroxyurea intake is the primary mechanism by which hydroxyurea modifies the pathogenesis of sickle cell disease
PubDate: Thu, 29 Feb 2024 00:00:00 +000
- Pneumatosis cystoides intestinalis with fatal air embolism after minor
blunt abdominal trauma in a 6-year-old girl undergoing hematopoietic stem
cell trasplant: case report and review of literature
Authors: Matteo Chinello; Olivia Chapin Arnone, Silvia Artusa, Giorgia Mazzuca, Elisa Bonetti, Virginia Vitale, Ada Zaccaron, Dario Raniero, Simone Cesaro
PubDate: Thu, 29 Feb 2024 00:00:00 +000
- FLT3 Mutated Acute Myeloid Leukemia after CD19 CAR-T Cells
Authors: Eugenio Galli; Filippo Frioni, Tanja Malara, Enrico Attardi, Silvia Bellesi, Stefan Hohaus, Simona Sica, Federica Sorà, Patrizia Chiusolo
Abstract: Chimeric Antigen Receptor T-cells have improved the life expectancy of severely pretreated patients with aggressive hematological cancers; for this reason, therapy-related myeloid leukemias are becoming of great concern in this field, despite their clonal phylogenesis and mutational landscape have not been fully explored yet. This case discusses a 33-year-old man with refractory large B-cell lymphoma, treated with Chimeric Antigen Receptor T-cell (CAR-T) therapy as the 7th line of treatment. Despite a persistent partial response, the patient developed therapy-related acute myeloid leukemia (t-AML) six months post-CAR-T, revealing pre-existing clonal hematopoiesis. The myeloid malignancy exhibited an unusual hypocellular/dysplastic pattern, progressing to an established blast phase with cytopenia. Treatment with demethylating agents and BCL2 inhibitors proved ineffective, leading to t-AML with hyperleukocytosis and FLT3-ITD gain, resulting in the patient's death. This case underscores the impact of severe pretreatment and bone marrow impairment in CAR-T-associated t-AML, emphasizing their role over insertional mutagenesis. Furthermore, it highlights the retention of classic therapy-related leukemia characteristics, including the potential for acquiring FLT3 mutations and displaying dysplastic morphology in these secondary leukemias.
PubDate: Thu, 29 Feb 2024 00:00:00 +000
- Internal Medicine Ward with Hematological Skills for the Treatment of
Complications Suffered by Hematological Patients on Therapy: Experience of
Villa Betania Hospital inRome
Authors: alessandro andriani; Laura Marchetti, Fabio Rossi, Silvia Raja, Maria Antonietta Perretti, Umberto Recine
PubDate: Thu, 29 Feb 2024 00:00:00 +000
- Probable autoimmune lymphoproliferative syndrome with monogenic lupus due
to KRAS mutation - A rare encounter
Authors: Amiya Nayak; Pratyusha Gudapati, Swapnil Tripathi, Jasmita Dass, Mukul Aggarwal, Pradeep Kumar
Abstract: Autoimmune lymphoproliferative syndrome (ALPS) is a disease characterized by dysfunction of the T lymphocyte apoptotic pathways, mostly due to dysfunctional FAS mediated signaling. However few cases can also occur independent of FAS pathway alteration. ALPS is characterized by various immuno-hematological manifestations. Monogenic lupus is an evolving entity, which describes the etiologic role of single gene modulation in systemic lupus erythematosus. In this manuscript, we describe a case of probable ALPS with monogenic lupus caused by a novel mutation in the KRAS gene.
PubDate: Thu, 29 Feb 2024 00:00:00 +000
- PLASMABLASTIC LYMPHOMA. A STATE-OF-THE-ART REVIEW (1)
Authors: Michele Bibas
Abstract: The objective of this two-part study is to present current and comprehensive understanding on the diagnosis and management of plasmablastic lymphoma. The first section, as presented in this paper, is on the study of epidemiology, etiology, clinopathological characteristics, differential diagnosis, prognostic variables, and the impact of plasmablastic lymphoma on specific populations. Plasmablastic lymphoma (PBL), a rare and aggressive form of lymphoma. Previous and modern studies have demonstrated a significant association between the human immunodeficiency virus (HIV) and the development of the disease. The limited occurrence of PBL contributes to a lack of comprehensive understanding regarding the molecular mechanisms involved in its etiology. Consequently, the diagnostic procedure for PBL poses a significant difficulty. Among the group of CD20-negative large B-cell lymphomas, PBL can be correctly diagnosed by identifying its exact clinical characteristics, anatomical location, and morphological characteristics. PBL cells do not express CD20 or PAX5 but possess plasmacytic differentiation markers such as CD38, CD138, MUM1/IRF4, Blimp1, and XBP1. PBL must be distinguished from other B-cell malignancies that lack the CD20 marker, including primary effusion lymphoma, anaplastic lymphoma kinase-positive large B-cell lymphoma, and large B-cell lymphoma (LBCL). This condition is frequently associated with infections caused by the Epstein-Barr virus and genetic alterations involving the MYC gene. Despite advances in our comprehension of this disease, the prognosis remains dismal, resulting in a low overall survival rate, although recent reports suggest an apparent tendency towards substantial improvement.
PubDate: Mon, 01 Jan 2024 00:00:00 +000
- CAR-T CELL THERAPY IN B-CELL ACUTE LYMPHOBLASTIC LEUKEMIA
Authors: Ugo Testa; Prof. Sica, Dr. Pelosi, Dr. Germana Castelli, Prof. Leone
Abstract: Treatment of refractory and relapsed (R/R) B acute lymphoblastic leukemia (B-ALL) is an unmet medical need in both children and adults. Studies carried out in the last two decades have shown that autologous T cells engineered to express a chimeric antigen receptor (CAR-T) represent an effective technique for treating these patients. Antigens expressed on B-cells, such as CD19, CD20, and CD22, represent targets suitable for treating patients with R/R B-ALL. CD19 CAR-T cells induce a high rate (80-90%) of complete remissions in both pediatric and adult R/R B-ALL patients. However, despite this impressive rate of responses, about half of responding patients relapse within 1-2 years after CAR-T cell therapy. Allo-HSCT after CAR-T cell therapy might consolidate the therapeutic efficacy of CAR-T and increase long-term outcomes; however, not all the studies that have adopted allo-HSCT as a consolidative treatment strategy have shown a benefit deriving from transplantation. For B-ALL patients who relapse early after allo-HSCT or those with insufficient T-cell numbers for an autologous approach, using T cells from the original stem cell donor offers the opportunity for the successful generation of CAR-T cells and for an effective therapeutic approach. Finally, recent studies have introduced allogeneic CAR-T cells generated from healthy donors or unmatched, which are opportunely manipulated with gene editing to reduce the risk of immunological incompatibility, with promising therapeutic effects. Keywords: CAR T; Acute Lymphoid Leukemia; Allogeneic CAR-T; Autologous CAR-T.
PubDate: Mon, 01 Jan 2024 00:00:00 +000
- CAR-T CELL THERAPY FOR FOLLICULAR LYMPHOMAS
Authors: Ugo Testa; Francesco D'Alò, Elvira Pelosi, Germana Castelli, Giuseppe Leone
Abstract: Follicular lymphoma is the second most diagnosed lymphoma in Western Europe. Significant advancements have considerably improved the survival of FL patients. However, 10-20% of these patients are refractory to standard treatments, and most of them will relapse. The treatment of follicular lymphoma patients with multiply relapsed or refractory disease represents an area of high-unmet needing new treatments with stronger efficacy. Chimeric antigen receptor (CAR)-T cell therapy targeting B-cell antigens, such as CD19 or CD20, is emerging as an efficacious treatment for R/R follicular lymphoma patients, particularly for those with early relapse and refractory to alkylating agents and to anti-CD20 monoclonal antibodies, resulting in a high rate of durable responses in a high proportion of patients.
PubDate: Mon, 01 Jan 2024 00:00:00 +000
- THALIDOMIDE AMELIORATES ERYTHROPOIESIS AND IRON HOMEOSTASIS IN
TRANSFUSION-DEPENDENT β-THALASSEMIA
Authors: Kun Yang; Jian Xiao
Abstract: Thalidomide is a therapeutic option for patients with β-thalassemia by increasing fetal hemoglobin and thereby reducing the requirement for blood transfusions. However, information on changes in erythropoiesis and iron homeostasis during thalidomide treatment is lacking. This study investigated the effects of thalidomide treatment on hematologic, erythropoietic, and iron-status parameters in 22 patients with transfusion-dependent β-thalassemia (TDT). Thalidomide significantly improved anemia endpoints, including increases in hemoglobin (p<0.001), red blood cells (p<0.001), and hematocrit (p<0.001), as well as reducing erythropoietin levels (p=0.033) and ameliorating erythropoiesis. Thalidomide treatment significantly reduced serum iron levels (p=0.018) and transferrin saturation (p=0.039) and increased serum transferrin levels (p=0.030). Thalidomide had no observed effect on serum ferritin or hepcidin, but changes in hepcidin(r=0.439, p=0.041) and serum iron (r=−0.536, p=0.010) were significantly correlated with hemoglobin increment. This comprehensive study indicates that thalidomide treatment can ameliorate erythropoiesis and iron homeostasis in patients with TDT, thus supporting the effectiveness of this drug.
PubDate: Mon, 01 Jan 2024 00:00:00 +000
- HAPLOIDENTICAL TRANSPLANT WITH POST-TRANSPLANT CYCLOPHOSPHAMIDE FOR ACUTE
MYELOID LEUKAEMIA AND MYELODYSPLASTIC SYNDROMES PATIENTS: THE ROLE OF
PREVIOUS LINES OF THERAPY.
Authors: Daniele Avenoso; Fabio Serpenti, Liron Barnea Slonim, Styliani Bouziana, Francesco Dazzi, Guy Hannah, Michelle Kenyon, Varun Mehra, Austin Kulasekararaj, Pramila Krishnamurthy, Mili Naresh Shah, Sharon Lionel, Antonio Pagliuca, Victoria Potter
Abstract: Background: Allogeneic haematopoietic stem-cell transplant is a potentially curative option for high-risk acute myeloid leukaemia (AML) and myelodysplastic syndrome (MDS) patients. Post-transplant cyclophosphamide administration allows for selection of haploidentical donors in patients who are eligible for the procedure, but do not have a fully matched donor, since it can overcome the HLA barrier. There is still an active debate on whether intensification of the conditioning regimen is necessary with haploidentical donors when peripheral blood stem cells are used as the source of the graft. Herein we report our decennial experience of haploidentical stem-cell transplant using peripheral blood stem cells at King’s College Hospital. Objectives: The primary objective was to evaluate overall survival (OS) for patients with less than two previous lines of therapy. Secondary objectives were total OS, OS according to cytomegalovirus (CMV) reactivation, incidence of transplant-related mortality (TRM), graft-versus-host disease (GVHD) and GVHD-relapse-free survival (GRFS). Results: One-year and three-year total OS were 62% and 43%, respectively, with a median OS of 22 months. One-year and three-year OS for patients with ≤2 and in patients with >2 previous lines of therapy were 72% and 55%, and 60% and 22%, respectively (p-value=0.04). The median OS in patients with >2 previous lines of therapy and ≤2 lines of therapy was 16 and 49 months, respectively. Cumulative incidence (CI) of relapse was 25% with a median time to relapse of 5 months (range 1 – 38 months). Conclusions: Haploidentical haematopoietic stem-cell transplant is potentially curative in chemo-sensitive AML and MDS and offers a high rate of prolonged remission. Our cohort further confirms the role of consolidative haploidentical transplant in patients in complete remission and highlights that patients with heavily pre-treated disease may not benefit from this strategy.
PubDate: Mon, 01 Jan 2024 00:00:00 +000
- ASSOCIATION BETWEEN LEUKEMIC EVOLUTION AND UNCOMMON CHROMOSOMAL
ALTERATIONS IN PEDIATRIC MYELODYSPLASTIC SYNDROME
Authors: Viviane Lamim Lovatel; Beatriz Ferreira da Silva , Eliane Ferreira Rodrigues , Maria Luiza Rocha da Rosa Borges , Rita de Cássia Barbosa Tavares, Ana Paula Silva Bueno, Elaine Sobral da Costa, Terezinha de Jesus Marques , Teresa de Souza Fernandez
Abstract: Background and objective: Pediatric myelodysplastic syndrome (pMDS) is a group of rare clonal neoplasms with a difficult diagnosis and risk of progression to acute myeloid leukemia (AML). The early stratification in risk groups is essential to choosing the treatment and indication for allogeneic hematopoietic stem cell transplantation (HSCT). According to the Revised International Prognostic Scoring System, cytogenetic analysis has demonstrated an essential role in diagnosis and prognosis. In pMDS, abnormal karyotypes are present in 30-50% of the cases. Monosomy 7 is the most common chromosomal alteration associated with poor prognosis. However, the rarity of specific cytogenetic alterations makes its prognosis uncertain. Thus, this study aimed to describe uncommon cytogenetic alterations in a cohort of 200 pMDS patients and their association with evolution to AML. Methods: The cytogenetic analysis was performed in 200 pMDS patients by G-banding and fluorescence in situ hybridization between 2000 to 2022. Results: Rare chromosome alterations were observed in 7.5% (15/200) of the cases. These chromosome alterations were divided into four cytogenetic groups: hyperdiploidy, biclonal chromosomal alterations, translocations, and uncommon deletions, which represented 33.3%, 33.3%, 20%, and 13.3%, respectively. Most of these patients (10/15) were classified with advanced MDS (MDS-EB and MDS/AML) and the initial subtype was present in five patients (RCC). The leukemic evolution was observed in 66.66% (10/15) of the patients. Most patients had poor clinical outcomes and they were indicated for HSCT. Conclusion: The study of uncommon cytogenetic alterations in pMDS is important to improve the prognosis and guide early indication of HSCT. Keywords: Pediatric MDS; Leukemic evolution; rare chromosomal altwerations; HSCT, Children
PubDate: Mon, 01 Jan 2024 00:00:00 +000
- CAN WE PREDICT INCIPIENT DIABETES MELLITUS IN PATIENTS WITH TRANSFUSION
Authors: Vincenzo De Sanctis; Ashraf Soliman, Shahina Daar, Ploutarchos Tzoulis, Christos Kattamis
Abstract: Background: Prediabetes and diabetes mellitus (DM) are complications in adult patients with transfusion dependent β-thalassemia (β-TDT), with their incidence increasing with age. Objective: This retrospective observational study describes the glycemic trajectories and evaluates predictive indices of β-cell function and insulin sensitivity/resistance in β-TDT patients with prediabetes, both in a steady state and during 3-h oral glucose tolerance test (OGTT), in order to identify patients at high risk for incipient diabetes. Setting: The study was mainly conducted at the Pediatric and Adolescent Outpatient Clinic, Quisisana Hospital, Ferrara (Italy) in collaboration with thalassemia referring centers across Italy. Patients: The study included 11 β-TDT (aged 15.11- 31.10 years) with history of prediabetes. Methods: The ADA criteria for the diagnosis of glucose dysregulation were adopted. Investigations included evaluation of plasma glucose levels and insulin secretion, analysis of glycemic trajectories and indices of β-cell function and insulin sensitivity/resistance assessed in steady state and during OGTT. Results: The duration of progression from prediabetes to DM, expressed in years, showed a positive direct correlation with corrected insulin response (CIR-30 = r: 0.7606, P: 0.0065), insulinogenic index (IGI 0-120 = r: 0.6121, P:0.045), oral disposition index (oDI = r: 0.7119, P:0.013), insulin growth factor-1 (IGF-1= r: 0.6246, P: 0.039) and an inverse linear correlation with serum ferritin (SF = r: -0.7197, P: 0.012). Conclusions: Progressive β-cell failure, peripheral resistance to the action of insulin and reduction of oDI were the principal factors responsible for the progression from prediabetes to incipient DM.
PubDate: Mon, 01 Jan 2024 00:00:00 +000
- SAFETY OF BRONCHOALVEOLAR LAVAGE IN HEMATOLOGICAL PATIENTS WITH
THROMBOCYTOPENIA – A RETROSPECTIVE COHORT STUDY
Authors: Ivan GUR; Roei Tounek, Yaniv Dotan, Elite Vainer Evgrafov, Stav Rakedzon, Eyal Fuchs
Abstract: Background Hospitalized hematological patients often require bronchoalveolar lavage (BAL). Paucity of evidence exists as to the potential risks in patients with very-severe thrombocytopenia (VST). Methods This retrospective-cohort study included adult hematological in-patients with VST, defined as platelets<20x103/μL, undergoing BAL during 2012-2021. Mechanically-ventilated patients or those with known active bleeding were excluded. Primary outcomes included major bleeding halting the BAL or deemed significant by the treating physician, need for any respiratory support other than low flow O2 or death within 24 hours. Any other bleeding were recorded as secondary outcomes. Results Of the 507 patients included in the final analysis, the 281 patients with VST had lower hemoglobin (Md=0.3, p=0.003), longer prothrombin-time (Md=0.7s, p=0.025), higher chances of preprocedural platelet transfusion (RR 3.68, 95%CI[2.86,4.73]), and only one primary-outcome event (death of septic shock 21h postprocedurally) - compared with 3 (1.3%) events (two bleedings halting procedure and one need for non-invasive-ventilation) in patients with platelets ≥20x103/μL (p=0.219). Risk of minor, spontaneously resolved bleeding was higher (RR=3.217, 95%CI[0.919,11.262]) in patients with VST (4.3% vs 1.3%, p=0.051). No association was found between any of the complications recorded and preprocedural platelets, age, aPTT, PT, hematological status, or platelet transfusion. Conclusions This data suggests BAL to be safe even when platelet counts are <20x103/μL.
PubDate: Mon, 01 Jan 2024 00:00:00 +000
- Prof, Bruno Bizzi Obituary
Authors: Valerio De Stefano
Abstract: x
PubDate: Mon, 01 Jan 2024 00:00:00 +000
- Seroprevalence of transfusion-transmissible infections among family
replacement donors and voluntary non-remunerated blood donors during the
COVID-19 pandemic in sub Saharan Africa
Authors: Macoura Gadji; Youssou Bamar Gueye, David Motto, Saliou Diop
Abstract: Introduction : According to WHO, regular, voluntary, unpaid blood donors are the safest group of donors, as they have the lowest prevalence of blood transmitted infections. However, family/replacement blood donors is widely used in sub Saharan Africa and this practice was exacerbated during the COVID 19 pandemic. This study aimed to compare the seroprevalence of infectious markers in family replacement blood donors and voluntary non-remunerated blood donors during the COVID 19 pandemic in a country of sub Saharan Africa. Materials and Methods Blood donors received at the National Centre of Blood Transfusion (NBTC) of Dakar from August 1st to October 31th 2021, were included in this study. All donors completed a pre-donation questionnaire. Donors identity, epidemiological parameters, reasons for donation and laboratory results were collected in the Inlog® software of the NBTC. The serological tests for HBV, HIV and HCV were performed with chemiluminescence technology. The Rapid Plasma Reagent test was used to find out treponemal antibodies. The determination of ABO and Rh blood groups was performed using monoclonal antisera following classical hemagglutination test on a plate. Results A total of 5002 donors were collected during this COVID-19 pandemic period. Blood family/replacement donors represented 54.0% and new voluntary donors represented 52.6%. Comparison of HIV, HCV and syphilis markers seroprevalence showed no statistically significant difference between new voluntary donors and new family replacement donors (p>0.05). However, for HBV the seroprevalence was significantly higher in new family replacement donors (p=0,002). Conclusion The proper supply of blood was impacted by the COVID-19 pandemic meanwhile replacement donations had contributed to limiting the damage observed with blood shortages. However, the significant differences noted on the seroprevalences of transfusion-transmissible infections between voluntary non-paid donors and family/replacement donors strengthens WHO recommendations for the selection of volunteer non-paid donors to lower transfusion-transmissible HBV in sub Saharan Africa.
PubDate: Mon, 01 Jan 2024 00:00:00 +000
- How we prevented an anti-P1 mediated hemolytic transfusion reaction
Authors: Beatrice Borsellino; Tiziano Martini, Rino Biguzzi, Irene Francesconi, Maria Federica Currà, Sabrina Lelli
PubDate: Mon, 01 Jan 2024 00:00:00 +000
- Hemophagocytic lymphohistiocytosis secondary to refractory acute myeloid
leukemia resolved after second line treatment with azacitidine plus
venetoclax
Authors: Claudio Fozza
Abstract: Hemophagocytic lymphohistiocytosis (HLH), also defined as hemophagocytic syndrome (HPS), represents a potentially life-threatening hyperinflammatory syndrome, characterized by impaired function of cytotoxic T lymphocytes, natural killer cells and macrophages. The main clinical features of HLH are prolonged fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, hyperferritinemia and hemophagocytosis in bone marrow, liver, spleen or lymph nodes. Secondary HLH typically occurs in conjunction with severe infections, malignancies or autoimmune disorders and intensive chemotherapy, potentially complicating treatment of acute myeloid leukemia (AML) in around 10% of cases. Herein we report for the first time a case of HLH secondary to refractory/relapsed AML resolved after a second line treatment with azacitidine plus venetoclax, thus offering a new potential therapeutic perspective in the context of a life-threatening clinical scenario.
PubDate: Mon, 01 Jan 2024 00:00:00 +000
- A case of central venous catheter-related Candida parapsilosis fungemia
evolved to disseminated infection in a neutropenic patient with blast
crisis of chronic myeloid leukemia.
Authors: Elena Amabile; Matteo Totaro, Luca Cappelli, Clara Minotti, Alessandra Micozzi
Abstract: Central venous catheter-related infections are of particular importance in onco-hematological patients. Candida parapsilosis is generally reported as a mild pathogen, however it is able to effectively colonize intravascular devices and potentially give rise to sustained fungemias. Here we report a case of invasive, potentially lethal C. parapsilosis disseminated infection in a neutropenic patient affected by chronic myeloid leukemia with blast crisis. We underline the importance of removing the central venous catheter as potential source of infection as soon as possible during the course of candidemia, and not replacing it with other polyurethan intravascular devices, which pose a risk for the maintenance of the fungemia despite the administration of the best antifungal therapy available.
PubDate: Mon, 01 Jan 2024 00:00:00 +000
- Successful Bridging to Allogeneic Transplantation With Valemetostat in Two
Refractory/relapsed Peripheral T-cell lymphoma patients
Authors: Gianmarco Bagnato; Vittorio Stefoni, Alessandro Broccoli, Lisa Argnani, Cinzia Pellegrini, Beatrice Casadei, Francesca Bonifazi, Pier Luigi Zinzani
Abstract: We report the case of 2 patients with relapsed/refractory peripheral T-cell lymphoma treated with valemetostat tosilate, a selective dual inhibitor of histone-lysine N-methyltransferases enhancer of zeste homolog 1 and 2, and subsequently bridged to allogeneic stem cell transplantation. Valemetostat led to a quick response and was well tolerated, offering as a promising bridge therapy to transplantation for patients with relapsed/refractory peripheral T-cell lymphoma which is still an unmet medical need.
PubDate: Mon, 01 Jan 2024 00:00:00 +000
