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Publisher: Elsevier   (Total: 3030 journals)

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Showing 1 - 200 of 3030 Journals sorted alphabetically
AASRI Procedia     Open Access   (Followers: 15)
Academic Pediatrics     Hybrid Journal   (Followers: 20, SJR: 1.402, h-index: 51)
Academic Radiology     Hybrid Journal   (Followers: 16, SJR: 1.008, h-index: 75)
Accident Analysis & Prevention     Partially Free   (Followers: 79, SJR: 1.109, h-index: 94)
Accounting Forum     Hybrid Journal   (Followers: 22, SJR: 0.612, h-index: 27)
Accounting, Organizations and Society     Hybrid Journal   (Followers: 27, SJR: 2.515, h-index: 90)
Achievements in the Life Sciences     Open Access   (Followers: 4)
Acta Anaesthesiologica Taiwanica     Open Access   (Followers: 5, SJR: 0.338, h-index: 19)
Acta Astronautica     Hybrid Journal   (Followers: 303, SJR: 0.726, h-index: 43)
Acta Automatica Sinica     Full-text available via subscription   (Followers: 3)
Acta Biomaterialia     Hybrid Journal   (Followers: 25, SJR: 2.02, h-index: 104)
Acta Colombiana de Cuidado Intensivo     Full-text available via subscription  
Acta de Investigación Psicológica     Open Access   (Followers: 2)
Acta Ecologica Sinica     Open Access   (Followers: 8, SJR: 0.172, h-index: 29)
Acta Haematologica Polonica     Free   (SJR: 0.123, h-index: 8)
Acta Histochemica     Hybrid Journal   (Followers: 3, SJR: 0.604, h-index: 38)
Acta Materialia     Hybrid Journal   (Followers: 196, SJR: 3.683, h-index: 202)
Acta Mathematica Scientia     Full-text available via subscription   (Followers: 5, SJR: 0.615, h-index: 21)
Acta Mechanica Solida Sinica     Full-text available via subscription   (Followers: 9, SJR: 0.442, h-index: 21)
Acta Oecologica     Hybrid Journal   (Followers: 9, SJR: 0.915, h-index: 53)
Acta Otorrinolaringologica (English Edition)     Full-text available via subscription   (Followers: 1)
Acta Otorrinolaringológica Española     Full-text available via subscription   (Followers: 3, SJR: 0.311, h-index: 16)
Acta Pharmaceutica Sinica B     Open Access   (Followers: 2)
Acta Poética     Open Access   (Followers: 4)
Acta Psychologica     Hybrid Journal   (Followers: 21, SJR: 1.365, h-index: 73)
Acta Sociológica     Open Access  
Acta Tropica     Hybrid Journal   (Followers: 5, SJR: 1.059, h-index: 77)
Acta Urológica Portuguesa     Open Access  
Actas Dermo-Sifiliograficas     Full-text available via subscription   (Followers: 4)
Actas Dermo-Sifiliográficas (English Edition)     Full-text available via subscription   (Followers: 3)
Actas Urológicas Españolas     Full-text available via subscription   (Followers: 3, SJR: 0.383, h-index: 19)
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Actualites Pharmaceutiques     Full-text available via subscription   (Followers: 5, SJR: 0.141, h-index: 3)
Actualites Pharmaceutiques Hospitalieres     Full-text available via subscription   (Followers: 4, SJR: 0.112, h-index: 2)
Acupuncture and Related Therapies     Hybrid Journal   (Followers: 4)
Ad Hoc Networks     Hybrid Journal   (Followers: 11, SJR: 0.967, h-index: 57)
Addictive Behaviors     Hybrid Journal   (Followers: 15, SJR: 1.514, h-index: 92)
Addictive Behaviors Reports     Open Access   (Followers: 5)
Additive Manufacturing     Hybrid Journal   (Followers: 7, SJR: 1.039, h-index: 5)
Additives for Polymers     Full-text available via subscription   (Followers: 20)
Advanced Drug Delivery Reviews     Hybrid Journal   (Followers: 120, SJR: 5.2, h-index: 222)
Advanced Engineering Informatics     Hybrid Journal   (Followers: 11, SJR: 1.265, h-index: 53)
Advanced Powder Technology     Hybrid Journal   (Followers: 16, SJR: 0.739, h-index: 33)
Advances in Accounting     Hybrid Journal   (Followers: 8, SJR: 0.299, h-index: 15)
Advances in Agronomy     Full-text available via subscription   (Followers: 15, SJR: 2.071, h-index: 82)
Advances in Anesthesia     Full-text available via subscription   (Followers: 24, SJR: 0.169, h-index: 4)
Advances in Antiviral Drug Design     Full-text available via subscription   (Followers: 3)
Advances in Applied Mathematics     Full-text available via subscription   (Followers: 6, SJR: 1.054, h-index: 35)
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Advances in Applied Microbiology     Full-text available via subscription   (Followers: 21, SJR: 1.286, h-index: 49)
Advances In Atomic, Molecular, and Optical Physics     Full-text available via subscription   (Followers: 16, SJR: 3.31, h-index: 42)
Advances in Biological Regulation     Hybrid Journal   (Followers: 4, SJR: 2.277, h-index: 43)
Advances in Botanical Research     Full-text available via subscription   (Followers: 3, SJR: 0.619, h-index: 48)
Advances in Cancer Research     Full-text available via subscription   (Followers: 26, SJR: 2.215, h-index: 78)
Advances in Carbohydrate Chemistry and Biochemistry     Full-text available via subscription   (Followers: 9, SJR: 0.9, h-index: 30)
Advances in Catalysis     Full-text available via subscription   (Followers: 5, SJR: 2.139, h-index: 42)
Advances in Cellular and Molecular Biology of Membranes and Organelles     Full-text available via subscription   (Followers: 12)
Advances in Chemical Engineering     Full-text available via subscription   (Followers: 24, SJR: 0.183, h-index: 23)
Advances in Child Development and Behavior     Full-text available via subscription   (Followers: 10, SJR: 0.665, h-index: 29)
Advances in Chronic Kidney Disease     Full-text available via subscription   (Followers: 8, SJR: 1.268, h-index: 45)
Advances in Clinical Chemistry     Full-text available via subscription   (Followers: 28, SJR: 0.938, h-index: 33)
Advances in Colloid and Interface Science     Full-text available via subscription   (Followers: 18, SJR: 2.314, h-index: 130)
Advances in Computers     Full-text available via subscription   (Followers: 16, SJR: 0.223, h-index: 22)
Advances in Developmental Biology     Full-text available via subscription   (Followers: 11)
Advances in Digestive Medicine     Open Access   (Followers: 4)
Advances in DNA Sequence-Specific Agents     Full-text available via subscription   (Followers: 5)
Advances in Drug Research     Full-text available via subscription   (Followers: 22)
Advances in Ecological Research     Full-text available via subscription   (Followers: 39, SJR: 3.25, h-index: 43)
Advances in Engineering Software     Hybrid Journal   (Followers: 25, SJR: 0.486, h-index: 10)
Advances in Experimental Biology     Full-text available via subscription   (Followers: 7)
Advances in Experimental Social Psychology     Full-text available via subscription   (Followers: 38, SJR: 5.465, h-index: 64)
Advances in Exploration Geophysics     Full-text available via subscription   (Followers: 3)
Advances in Fluorine Science     Full-text available via subscription   (Followers: 8)
Advances in Food and Nutrition Research     Full-text available via subscription   (Followers: 41, SJR: 0.674, h-index: 38)
Advances in Fuel Cells     Full-text available via subscription   (Followers: 14)
Advances in Genetics     Full-text available via subscription   (Followers: 15, SJR: 2.558, h-index: 54)
Advances in Genome Biology     Full-text available via subscription   (Followers: 11)
Advances in Geophysics     Full-text available via subscription   (Followers: 6, SJR: 2.325, h-index: 20)
Advances in Heat Transfer     Full-text available via subscription   (Followers: 18, SJR: 0.906, h-index: 24)
Advances in Heterocyclic Chemistry     Full-text available via subscription   (Followers: 8, SJR: 0.497, h-index: 31)
Advances in Human Factors/Ergonomics     Full-text available via subscription   (Followers: 22)
Advances in Imaging and Electron Physics     Full-text available via subscription   (Followers: 2, SJR: 0.396, h-index: 27)
Advances in Immunology     Full-text available via subscription   (Followers: 33, SJR: 4.152, h-index: 85)
Advances in Inorganic Chemistry     Full-text available via subscription   (Followers: 9, SJR: 1.132, h-index: 42)
Advances in Insect Physiology     Full-text available via subscription   (Followers: 3, SJR: 1.274, h-index: 27)
Advances in Integrative Medicine     Hybrid Journal   (Followers: 4)
Advances in Intl. Accounting     Full-text available via subscription   (Followers: 4)
Advances in Life Course Research     Hybrid Journal   (Followers: 7, SJR: 0.764, h-index: 15)
Advances in Lipobiology     Full-text available via subscription   (Followers: 1)
Advances in Magnetic and Optical Resonance     Full-text available via subscription   (Followers: 8)
Advances in Marine Biology     Full-text available via subscription   (Followers: 16, SJR: 1.645, h-index: 45)
Advances in Mathematics     Full-text available via subscription   (Followers: 10, SJR: 3.261, h-index: 65)
Advances in Medical Sciences     Hybrid Journal   (Followers: 5, SJR: 0.489, h-index: 25)
Advances in Medicinal Chemistry     Full-text available via subscription   (Followers: 5)
Advances in Microbial Physiology     Full-text available via subscription   (Followers: 4, SJR: 1.44, h-index: 51)
Advances in Molecular and Cell Biology     Full-text available via subscription   (Followers: 21)
Advances in Molecular and Cellular Endocrinology     Full-text available via subscription   (Followers: 10)
Advances in Molecular Toxicology     Full-text available via subscription   (Followers: 6, SJR: 0.324, h-index: 8)
Advances in Nanoporous Materials     Full-text available via subscription   (Followers: 3)
Advances in Oncobiology     Full-text available via subscription   (Followers: 3)
Advances in Organometallic Chemistry     Full-text available via subscription   (Followers: 15, SJR: 2.885, h-index: 45)
Advances in Parallel Computing     Full-text available via subscription   (Followers: 7, SJR: 0.148, h-index: 11)
Advances in Parasitology     Full-text available via subscription   (Followers: 7, SJR: 2.37, h-index: 73)
Advances in Pediatrics     Full-text available via subscription   (Followers: 20, SJR: 0.4, h-index: 28)
Advances in Pharmaceutical Sciences     Full-text available via subscription   (Followers: 14)
Advances in Pharmacology     Full-text available via subscription   (Followers: 13, SJR: 1.718, h-index: 58)
Advances in Physical Organic Chemistry     Full-text available via subscription   (Followers: 7, SJR: 0.384, h-index: 26)
Advances in Phytomedicine     Full-text available via subscription  
Advances in Planar Lipid Bilayers and Liposomes     Full-text available via subscription   (Followers: 3, SJR: 0.248, h-index: 11)
Advances in Plant Biochemistry and Molecular Biology     Full-text available via subscription   (Followers: 8)
Advances in Plant Pathology     Full-text available via subscription   (Followers: 5)
Advances in Porous Media     Full-text available via subscription   (Followers: 4)
Advances in Protein Chemistry     Full-text available via subscription   (Followers: 18)
Advances in Protein Chemistry and Structural Biology     Full-text available via subscription   (Followers: 17, SJR: 1.5, h-index: 62)
Advances in Psychology     Full-text available via subscription   (Followers: 56)
Advances in Quantum Chemistry     Full-text available via subscription   (Followers: 5, SJR: 0.478, h-index: 32)
Advances in Radiation Oncology     Open Access  
Advances in Small Animal Medicine and Surgery     Hybrid Journal   (Followers: 1, SJR: 0.1, h-index: 2)
Advances in Space Research     Full-text available via subscription   (Followers: 332, SJR: 0.606, h-index: 65)
Advances in Structural Biology     Full-text available via subscription   (Followers: 7)
Advances in Surgery     Full-text available via subscription   (Followers: 6, SJR: 0.823, h-index: 27)
Advances in the Study of Behavior     Full-text available via subscription   (Followers: 28, SJR: 1.321, h-index: 56)
Advances in Veterinary Medicine     Full-text available via subscription   (Followers: 14)
Advances in Veterinary Science and Comparative Medicine     Full-text available via subscription   (Followers: 12)
Advances in Virus Research     Full-text available via subscription   (Followers: 5, SJR: 1.878, h-index: 68)
Advances in Water Resources     Hybrid Journal   (Followers: 42, SJR: 2.408, h-index: 94)
Aeolian Research     Hybrid Journal   (Followers: 5, SJR: 0.973, h-index: 22)
Aerospace Science and Technology     Hybrid Journal   (Followers: 304, SJR: 0.816, h-index: 49)
AEU - Intl. J. of Electronics and Communications     Hybrid Journal   (Followers: 8, SJR: 0.318, h-index: 36)
African J. of Emergency Medicine     Open Access   (Followers: 4, SJR: 0.344, h-index: 6)
Ageing Research Reviews     Hybrid Journal   (Followers: 7, SJR: 3.289, h-index: 78)
Aggression and Violent Behavior     Hybrid Journal   (Followers: 390, SJR: 1.385, h-index: 72)
Agri Gene     Hybrid Journal  
Agricultural and Forest Meteorology     Hybrid Journal   (Followers: 15, SJR: 2.18, h-index: 116)
Agricultural Systems     Hybrid Journal   (Followers: 29, SJR: 1.275, h-index: 74)
Agricultural Water Management     Hybrid Journal   (Followers: 36, SJR: 1.546, h-index: 79)
Agriculture and Agricultural Science Procedia     Open Access  
Agriculture and Natural Resources     Open Access   (Followers: 1)
Agriculture, Ecosystems & Environment     Hybrid Journal   (Followers: 48, SJR: 1.879, h-index: 120)
Ain Shams Engineering J.     Open Access   (Followers: 5, SJR: 0.434, h-index: 14)
Air Medical J.     Hybrid Journal   (Followers: 3, SJR: 0.234, h-index: 18)
AKCE Intl. J. of Graphs and Combinatorics     Open Access   (SJR: 0.285, h-index: 3)
Alcohol     Hybrid Journal   (Followers: 9, SJR: 0.922, h-index: 66)
Alcoholism and Drug Addiction     Open Access   (Followers: 5)
Alergologia Polska : Polish J. of Allergology     Full-text available via subscription   (Followers: 1)
Alexandria Engineering J.     Open Access   (Followers: 1, SJR: 0.436, h-index: 12)
Alexandria J. of Medicine     Open Access  
Algal Research     Partially Free   (Followers: 7, SJR: 2.05, h-index: 20)
Alkaloids: Chemical and Biological Perspectives     Full-text available via subscription   (Followers: 3)
Allergologia et Immunopathologia     Full-text available via subscription   (Followers: 1, SJR: 0.46, h-index: 29)
Allergology Intl.     Open Access   (Followers: 5, SJR: 0.776, h-index: 35)
ALTER - European J. of Disability Research / Revue Européenne de Recherche sur le Handicap     Full-text available via subscription   (Followers: 6, SJR: 0.158, h-index: 9)
Alzheimer's & Dementia     Hybrid Journal   (Followers: 45, SJR: 4.289, h-index: 64)
Alzheimer's & Dementia: Diagnosis, Assessment & Disease Monitoring     Open Access   (Followers: 5)
Alzheimer's & Dementia: Translational Research & Clinical Interventions     Open Access   (Followers: 3)
American Heart J.     Hybrid Journal   (Followers: 45, SJR: 3.157, h-index: 153)
American J. of Cardiology     Hybrid Journal   (Followers: 47, SJR: 2.063, h-index: 186)
American J. of Emergency Medicine     Hybrid Journal   (Followers: 34, SJR: 0.574, h-index: 65)
American J. of Geriatric Pharmacotherapy     Full-text available via subscription   (Followers: 6, SJR: 1.091, h-index: 45)
American J. of Geriatric Psychiatry     Hybrid Journal   (Followers: 14, SJR: 1.653, h-index: 93)
American J. of Human Genetics     Hybrid Journal   (Followers: 32, SJR: 8.769, h-index: 256)
American J. of Infection Control     Hybrid Journal   (Followers: 25, SJR: 1.259, h-index: 81)
American J. of Kidney Diseases     Hybrid Journal   (Followers: 31, SJR: 2.313, h-index: 172)
American J. of Medicine     Hybrid Journal   (Followers: 48, SJR: 2.023, h-index: 189)
American J. of Medicine Supplements     Full-text available via subscription   (Followers: 3)
American J. of Obstetrics and Gynecology     Hybrid Journal   (Followers: 174, SJR: 2.255, h-index: 171)
American J. of Ophthalmology     Hybrid Journal   (Followers: 51, SJR: 2.803, h-index: 148)
American J. of Ophthalmology Case Reports     Open Access   (Followers: 2)
American J. of Orthodontics and Dentofacial Orthopedics     Full-text available via subscription   (Followers: 6, SJR: 1.249, h-index: 88)
American J. of Otolaryngology     Hybrid Journal   (Followers: 22, SJR: 0.59, h-index: 45)
American J. of Pathology     Hybrid Journal   (Followers: 23, SJR: 2.653, h-index: 228)
American J. of Preventive Medicine     Hybrid Journal   (Followers: 21, SJR: 2.764, h-index: 154)
American J. of Surgery     Hybrid Journal   (Followers: 32, SJR: 1.286, h-index: 125)
American J. of the Medical Sciences     Hybrid Journal   (Followers: 13, SJR: 0.653, h-index: 70)
Ampersand : An Intl. J. of General and Applied Linguistics     Open Access   (Followers: 5)
Anaerobe     Hybrid Journal   (Followers: 4, SJR: 1.066, h-index: 51)
Anaesthesia & Intensive Care Medicine     Full-text available via subscription   (Followers: 52, SJR: 0.124, h-index: 9)
Anaesthesia Critical Care & Pain Medicine     Full-text available via subscription   (Followers: 3)
Anales de Cirugia Vascular     Full-text available via subscription  
Anales de Pediatría     Full-text available via subscription   (Followers: 2, SJR: 0.209, h-index: 27)
Anales de Pediatría (English Edition)     Full-text available via subscription  
Anales de Pediatría Continuada     Full-text available via subscription   (SJR: 0.104, h-index: 3)
Analytic Methods in Accident Research     Hybrid Journal   (Followers: 2, SJR: 2.577, h-index: 7)
Analytica Chimica Acta     Hybrid Journal   (Followers: 38, SJR: 1.548, h-index: 152)
Analytical Biochemistry     Hybrid Journal   (Followers: 154, SJR: 0.725, h-index: 154)
Analytical Chemistry Research     Open Access   (Followers: 7, SJR: 0.18, h-index: 2)
Analytical Spectroscopy Library     Full-text available via subscription   (Followers: 10)
Anesthésie & Réanimation     Full-text available via subscription  
Anesthesiology Clinics     Full-text available via subscription   (Followers: 21, SJR: 0.421, h-index: 40)
Angiología     Full-text available via subscription   (SJR: 0.124, h-index: 9)
Angiologia e Cirurgia Vascular     Open Access  
Animal Behaviour     Hybrid Journal   (Followers: 143, SJR: 1.907, h-index: 126)
Animal Feed Science and Technology     Hybrid Journal   (Followers: 5, SJR: 1.151, h-index: 83)
Animal Reproduction Science     Hybrid Journal   (Followers: 5, SJR: 0.711, h-index: 78)
Annales d'Endocrinologie     Full-text available via subscription   (SJR: 0.394, h-index: 30)
Annales d'Urologie     Full-text available via subscription  
Annales de Cardiologie et d'Angéiologie     Full-text available via subscription   (SJR: 0.177, h-index: 13)
Annales de Chirurgie de la Main et du Membre Supérieur     Full-text available via subscription  
Annales de Chirurgie Plastique Esthétique     Full-text available via subscription   (Followers: 2, SJR: 0.354, h-index: 22)
Annales de Chirurgie Vasculaire     Full-text available via subscription   (Followers: 1)

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Journal Cover Acta Haematologica Polonica
  [SJR: 0.123]   [H-I: 8]   [0 followers]  Follow
    
  Free journal Free journal
   ISSN (Print) 0001-5814
   Published by Elsevier Homepage  [3030 journals]
  • Visceral varicella-zoster virus (VZV) infection as an underestimated
           differential diagnosis of acute abdomen in a patient after allogeneic
           hematopoietic stem cell transplantation
    • Authors: Julia Radoń-Proskura; Ninela Irga-Jaworska; Anna Malinowska; Jan Maciej Zaucha
      Abstract: Publication date: Available online 15 March 2017
      Source:Acta Haematologica Polonica
      Author(s): Julia Radoń-Proskura, Ninela Irga-Jaworska, Anna Malinowska, Jan Maciej Zaucha
      We report a case of 18-year-old male patient who 5.5 months after allogeneic hematopoietic stem cell transplant (HSCT) developed severe abdominal pain not responding to high dose of opioids. The pain was accompanied by gradually increasing activity of liver enzymes and bilirubin concentration. The patient had a history of acute GVHD and was on steroid taper. Importantly, he was also temporarily off standard acyclovir prophylaxis. Provisional diagnosis of acute cholecystitis was made, however, cholecystectomy did not improve patient's condition. Clinical picture of severe abdominal pain without clear surgical cause, resistant to high doses of opiates with increasing activity of liver enzymes was highly suspicious of visceral varicella zoster virus (VZV) reactivation. Immediate introduction of intravenous acyclovir led to full recovery and complete resolution of abdominal pain. We conclude that reactivation of latent VZV with absent or delayed occurrence of characteristic skin vesicles may still pose a diagnostic challenge resulting in delay of the proper diagnosis and start of life saving antiviral treatment. Severe intractable pain in HSCT recipients with increasing activity of liver enzymes should evoke high index of suspicion of the possible disseminated VZV and impose start of empirical treatment with high dose acyclovir.

      PubDate: 2017-03-16T04:40:07Z
      DOI: 10.1016/j.achaem.2017.02.001
       
  • Osoczowe stężenie cytruliny w klinicznej ocenie toksyczności jelitowej
           u chorych na nowotwory złośliwe poddanych chemioterapii wysokodawkowej
           [Acta Haematologica Polonica 2015; 46(S): 190–191]
    • Authors: J. Barzał; P. Rzepecki; M. Jaworska; E. Anuszewska
      Abstract: Publication date: Available online 11 March 2017
      Source:Acta Haematologica Polonica
      Author(s): J. Barzał, P. Rzepecki, M. Jaworska, E. Anuszewska


      PubDate: 2017-03-16T04:40:07Z
      DOI: 10.1016/j.achaem.2017.03.001
       
  • Role of HLA match on results of hematopoietic stem cell transplantations
           from unrelated donors in children with acute leukemia and bone marrow
           failure syndromes
    • Authors: Jan Styczyński; Robert Dębski; Anna Krenska; Krzysztof Czyżewski; Natalia Bartoszewicz; Ewa Demidowicz; Ninela Irga-Jaworska; Elżbieta Drożyńska; Marcin Płonowski; Maryna Krawczuk-Rybak; Tomasz Ociepa; Tomasz Urasiński; Mariusz Wysocki
      Abstract: Publication date: Available online 28 February 2017
      Source:Acta Haematologica Polonica
      Author(s): Jan Styczyński, Robert Dębski, Anna Krenska, Krzysztof Czyżewski, Natalia Bartoszewicz, Ewa Demidowicz, Ninela Irga-Jaworska, Elżbieta Drożyńska, Marcin Płonowski, Maryna Krawczuk-Rybak, Tomasz Ociepa, Tomasz Urasiński, Mariusz Wysocki
      Background In case of the lack of matched family donors (MFD), hematopoietic stem cell transplantation (HSCT) from unrelated donor (UD) is an established procedure for many acquired and congenital disorders of the hematopoietic system, including malignancies and bone marrow failure (BMF) syndromes. Objective The analysis of the results of HSCT in patients with acute leukemia or BMF syndromes from UDs with respect to human leukocyte antigen (HLA) match. Patients and methods A total number of 97 of HSCT from UDs performed in single center between 2007 and 2015 in children and adolescents with acute lymphoblastic (ALL) or myeloblastic leukemia (AML) and BMF syndromes were included into this analysis. HLA match between donor and recipient was analyzed at the allele level and classified as 10/10, 9/10 or 8/10. Data were compared to results of 56 MFD-HSCTs. Probability of overall survival (pOS) was given for 3-year and 1-year (as required by JACIE standards) time periods. Results The mean survival for all patients estimated by Kaplan–Meier method was 4.8 years (95%CI=4.1–5.5 years). The 3-year pOS after all UD-HSCT was 0,60±0,05, and with respect to 10/10, 9/10 and 8/10 HLA match: 0,61±0,06; 0,59±0,09 and 0,60±0,22, respectively (ns). In patients with AML, 3-year pOS reached 52%, 60% and 60%, respectively. In patients with ALL, 3-year pOS was 73% and 62% (ns) for 10/10 and 9/10 HLA match, respectively, while for BMF syndromes 86% and 57% (ns), respectively. Conclusion Current data suggest that results of mismatched and matched UD-HSCT in children with acute leukemia might be comparable.

      PubDate: 2017-03-01T20:47:40Z
      DOI: 10.1016/j.achaem.2017.01.002
       
  • Szczepienia ochronne u chorych dorosłych po przeszczepieniu komórek
           krwiotwórczych – zalecenia sekcji do spraw zakażeń PALG
    • Authors: Agnieszka Piekarska; Sebastian Giebel; Grzegorz Władysław Basak; Jarosław Dybko; Kazimierz Hałaburda; Iwona Hus; Ewa Karakulska-Prystupiuk; Beata Jakubas; Patrycja Mensah-Glanowska; Piotr Rzepecki; Agnieszka Wierzbowska; Lidia Gil
      Abstract: Publication date: Available online 16 February 2017
      Source:Acta Haematologica Polonica
      Author(s): Agnieszka Piekarska, Sebastian Giebel, Grzegorz Władysław Basak, Jarosław Dybko, Kazimierz Hałaburda, Iwona Hus, Ewa Karakulska-Prystupiuk, Beata Jakubas, Patrycja Mensah-Glanowska, Piotr Rzepecki, Agnieszka Wierzbowska, Lidia Gil
      Infections are the most serious complications in patients undergoing hematopoietic cell transplantation (HCT). Vaccinations occur to be undeniably one of the most important prophylactic strategies and are routinely recommended in the post-transplantation period. Their use reduces the incidence of infections and infection-related mortality. In this paper, we present the current guidelines for active immunization after HCT for the Polish patient population as worked out by the Polish Adult Leukemia Group (PALG). The guidelines include the recommended vaccines, the optional vaccines and vaccination with live pathogens. The management in specific situations such as graft versus host disease and for HCT recipients travelling to exotic countries is clarified. We also present the proposal for organization of the vaccination program in transplant centers. The improvement in realization of the vaccination protocol is one of the key aspects of post-transplantation care in Poland.

      PubDate: 2017-02-23T16:30:29Z
      DOI: 10.1016/j.achaem.2017.01.003
       
  • Alloimmunologiczna małopłytkowość noworodków
    • Authors: Irmina Nowak; Weronika Kubiak-Prałat; Marcin Minta; Marta Szymankiewicz; Janusz Gadzinowski; Dawid Szpecht
      Abstract: Publication date: Available online 16 February 2017
      Source:Acta Haematologica Polonica
      Author(s): Irmina Nowak, Weronika Kubiak-Prałat, Marcin Minta, Marta Szymankiewicz, Janusz Gadzinowski, Dawid Szpecht
      Fetal/neonatal alloimmune thrombocytopenia (FNAIT) is defined as a platelet count <150,000/μL due to reaction between maternal antibodies and antigens located on thrombocytes of the fetus/neonate. Such a kind of pathology occurs when a mother does not possess specific human platelet antigens (HPA), which are inherited as an infant from the father. HPA-1a is an antigen that most often causes FNAIT in the Caucasian race. Frequency of FNAIT has been estimated as 1:350–1:5000. In the pathogenesis of FNAIT, the mother organism is immunized and produces alloantibodies from which IgG pass through the placenta, enter fetal circulatory system and cause platelet destruction. It usually takes place at the end of the second trimester. FNAIT can occur in the first pregnancy; nonetheless, it is more probable and connected with higher severity in subsequent gestations. It is caused by the fact that immunization usually takes place during the first labour, which enables production of alloantibodies in the next pregnancies. Nevertheless, other ways of immunization are also possible, which ensures that FNAIT cases in the first pregnancy are not casuistic and occur more often than RhD hemolytic disease of the newborn. Thrombocytopenia leads to coagulation disorders, and consequently to bleedings. FNAIT can be manifested not only by some petechiae on the skin, but also by severe hemorrhages in the body cavities and gastrointestinal tract or by intracranial hemorrhages (ICH). In case the symptoms are presented by an infant, platelet transfusions are performed, and IVIG (intravenous immunoglobulin) therapy is administered.

      PubDate: 2017-02-23T16:30:29Z
      DOI: 10.1016/j.achaem.2017.01.004
       
  • Diagnosing Beta Thalassemia trait in a developing country
    • Authors: Shan-e- Rauf; Ghassan Umair Shamshad; Fareeha Mushtaq; Saleem Ahmed Khan; Nadir Ali
      Abstract: Publication date: Available online 9 February 2017
      Source:Acta Haematologica Polonica
      Author(s): Shan-e- Rauf, Ghassan Umair Shamshad, Fareeha Mushtaq, Saleem Ahmed Khan, Nadir Ali


      PubDate: 2017-02-10T01:06:52Z
      DOI: 10.1016/j.achaem.2017.01.001
       
  • Zakażenia wirusowe u dzieci po przeszczepieniu komórek krwiotwórczych:
           raport 2016 Polskiej Pediatrycznej Grupy ds. Zakażeń Polskiego
           Towarzystwa Onkologii i Hematologii Dziecięcej
    • Authors: Jan Styczyński; Krzysztof Czyżewski; Jowita Frączkiewicz; Małgorzata Salamonowicz; Olga Zając-Spychała; Agnieszka Zaucha-Prażmo; Jolanta Goździk; Patrycja Zalas-Więcek; Magdalena Dziedzic; Krzysztof Kałwak; Ewa Gorczyńska; Alicja Chybicka; Jacek Wachowiak; Jerzy Kowalczyk; Eugenia Gospodarek-Komkowska; Mariusz Wysocki
      Abstract: Publication date: Available online 19 January 2017
      Source:Acta Haematologica Polonica
      Author(s): Jan Styczyński, Krzysztof Czyżewski, Jowita Frączkiewicz, Małgorzata Salamonowicz, Olga Zając-Spychała, Agnieszka Zaucha-Prażmo, Jolanta Goździk, Patrycja Zalas-Więcek, Magdalena Dziedzic, Krzysztof Kałwak, Ewa Gorczyńska, Alicja Chybicka, Jacek Wachowiak, Jerzy Kowalczyk, Eugenia Gospodarek-Komkowska, Mariusz Wysocki
      Background Polish Pediatric Infectious Working Group of Polish Society of Pediatric Oncology and Hematology continues from 2012 the infections monitoring program in pediatric hematopoietic stem cell transplant (HSCT) and onco-hematology centers. Objective Epidemiological analysis of viral infections in children and adolescents undergoing HSCT in pediatric centers in 2012–2013 and 2014–2015. Methods Retrospective analysis of viral infections after 650 HSCT in children and adolescents. Results An increase in incidence in 2014–2015 was observed (60.6% vs 51.3%; OR=1.5; p=0.035) after allo-HSCT. Cumulative incidence after allo-HSCT (2012–2013 vs. 2014–2015) was: CMV – 28.0% vs. 29.2%, BKV – 18.5% vs. 22.8%, EBV – 15.5% vs. 24.3%, ADV – 9.5% vs. 5.2%, rotavirus – 9.1% vs. 5.6%, VZV – 2.6% vs. 1.1%, influenza – 0.9% vs. 3.4%, HHV6 – 0.9% vs. 1.5%, norovirus – 0% vs. 2.2%, RSV – 0% vs. 1.5%, parainfluenza – 0% vs. 0.7%, and MPV – 0% vs 0.4%. Infections after auto-HSCT occurred in 8 (10.5%) patients between 2012 and 2013 vs. 2 (2.6%) between 2014 and 2015. Cure rate after viral infections has increased (2012–2013 vs. 2014–2015) for: EBV – 90.7% vs. 100%, ADV – 93.8% vs. 100%, BKV – 94.2% vs. 96.8%, CMV – 94.6% vs. 98%, and remained 100% in infections with influenza, VZV, HHV6, rotavirus as well as in parainfluenza, RSV, and MPV. Decrease of deaths rate attributed to viral infections from 6.5% (2012–2013) to 0.7% (2014–2015) was observed after allo-HSCT. Conclusions We found epidemiological trends in viral infections after HSCT in children: increase in incidence after allo-HSCT (increase EBV, appearance of CARV) and decrease after auto-HSCT. Decrease of deaths attributed to viral infections was observed in the last period of time.

      PubDate: 2017-01-19T20:04:21Z
      DOI: 10.1016/j.achaem.2016.11.006
       
  • Wyniki stosowania nowych metod rehabilitacji u pacjentów z
           artropatią hemofilową
    • Authors: Janusz Zawilski; Adrian Dudek; Przemysław Lisiński
      Abstract: Publication date: Available online 19 January 2017
      Source:Acta Haematologica Polonica
      Author(s): Janusz Zawilski, Adrian Dudek, Przemysław Lisiński
      Advanced arthropathy mainly of the knee, hip or ankle joint occurs even in young adults with haemophilia. It has been proved that physical training increases isometric muscular strength and proprioceptive performance in haemophilia patients. The aim of this study was to present the new methods of physiotherapy process and their effect in patients with haemophilic arthropathy. Five subjects, aged from 32 to 42 years, with severe haemophilia A or B attended physiotherapy in a outpatient ambulatory setting over a 3-month period. The following treatment modalities were performed: walking on AlterG anti-gravity treadmill, deep penetrating electromagnetic stimulation (Salus Talent), manual physical therapy, mobilization and manipulation techniques, active muscle-strengthening exercises, post isometric relaxation (PIR) muscle energy techniques, as well as exercises for improvement of coordination, postural equilibrium and proprioception exercises using sensorimotor discs. The HJHS (Haemophilia Joint Health Score) has been used to assess the effectiveness of the treatment, VAS scale (Visual Analog Scale) to assess level of pain, TUG test (Timed Up and Go) to assess mobility as well as dynamic and static balance. Strength of the muscles acting on the joints improved, swelling of joints diminished and the level of pain decreased. An improvement of the dynamic and static balance was found as well. The range of motion did not change. Physiotherapy process did not provoke bleeding episodes in patients with haemophilic arthropathy included to the study.

      PubDate: 2017-01-19T20:04:21Z
      DOI: 10.1016/j.achaem.2016.11.005
       
  • Rekomendacje diagnostyczne i terapeutyczne dla przewlekłej białaczki
           limfocytowej w 2016 r – Raport Grupy Roboczej PTHiT i PALG-CLL
    • Authors: Tadeusz Robak; Iwona Hus; Krzysztof Giannopoulos; Jerzy Błoński; Krzysztof Jamroziak; Jacek Roliński; Piotr Smolewski; Dariusz Wołowiec
      Pages: 169 - 183
      Abstract: Publication date: Available online 16 August 2016
      Source:Acta Haematologica Polonica
      Author(s): Tadeusz Robak, Iwona Hus, Krzysztof Giannopoulos, Jerzy Błoński, Krzysztof Jamroziak, Jacek Roliński, Piotr Smolewski, Dariusz Wołowiec
      The management of patients with chronic lymphocytic leukaemia (CLL) is currently undergoing improvements, particularly because of novel therapies. Purine analogs based immuno-chemotherapy, particularly fludarabine combined with cyclophosphamide and rituximab (FCR), is still the current standard of care for first line therapy in younger, fit patients. However, its use in older, co-morbid patients is limited, particularly due to high toxicity. Recently, in these patients’ population, chlorambucil and anti CD20 monoclonal antibodies (rituximab, obinutuzumab or ofatumumab) are recommended as the first-line treatment. In addition, in 2014, two novel agents targeting the B cell receptor (BCR) signaling pathway, ibrutinib and idelalisib, were approved for patients with 17p deletion and/or p53 mutations and in the relapsed situation because of high efficacy and a favorable toxicity. Subsequently, ibrutinib has been approved to treat CLL patients regardless of their treatment history. Thus far it is recommended to treat patients with these agents until progression or unacceptable toxicity. The BCL-2 antagonist venetoclax is another oral drug with very promising preliminary data in patients refractory to immunochemotherapy as well as patients harboring del 17p. In 2014, the PTH i T and PALG-CLL group defined guidelines for the diagnosis, prognosis and treatment of CLL. In this article, we present updated recommendations for therapy of CLL.

      PubDate: 2016-08-20T04:57:13Z
      DOI: 10.1016/j.achaem.2016.08.003
      Issue No: Vol. 47, No. 3 (2016)
       
  • Zalecenia dotyczące oceny schorzeń współistniejących u chorych na
           przewlekłą białaczkę szpikową w procesie wyboru inhibitora kinaz
           tyrozynowych
    • Authors: Tomasz Sacha; Sebastian Szmit; Dorota Zozulińska-Ziółkiewicz; Witold Prejzner; Joanna Góra-Tybor
      Pages: 184 - 196
      Abstract: Publication date: Available online 3 July 2016
      Source:Acta Haematologica Polonica
      Author(s): Tomasz Sacha, Sebastian Szmit, Dorota Zozulińska-Ziółkiewicz, Witold Prejzner, Joanna Góra-Tybor
      Treatment of chronic myeloid leukemia with tyrosine kinase inhibitors (TKI) is very effective. The vast majority of patients achieve deep and long-lasting therapeutic responses including those on molecular level. Understanding the adverse effects of TKIs is of great importance during life-long therapy. The safety profile of TKI should be considered especially when used in patients suffering from various co-morbidities. In this paper, treatment recommendations for patients suffering from diabetes mellitus, atherosclerosis with circulation disturbances in peripheral arteries, congestive heart failure, pulmonary hypertension and pleural effusions are presented. Drug interactions between TKIs and medicaments used in the treatment of the above-mentioned co-morbidities are discussed.

      PubDate: 2016-07-05T04:45:15Z
      DOI: 10.1016/j.achaem.2016.06.001
      Issue No: Vol. 47, No. 3 (2016)
       
  • Stres siateczki śródplazmatycznej i stres oksydacyjny w ostrych
           białaczkach szpikowych
    • Authors: Justyna Chlebowska
      Pages: 197 - 204
      Abstract: Publication date: Available online 6 August 2016
      Source:Acta Haematologica Polonica
      Author(s): Justyna Chlebowska
      Use of differentiation-inducing agents (all-trans retinoic acid and arsenic trioxide) that degradate fusion PML-RARα receptor have revolutionized management of acute promyelocytic (APL) leukemia in 2008. However, despite significant advances in the treatment of APL, the cure rates of patients suffering with other acute myeloid leukemia (AML) subtypes are still not satisfying. Abnormal reactive oxygen species levels and constitutive expression of ER stress marker proteins are characteristic of AML. AML patients with activated unfolded protein response and increased ER chaperone levels showed suppressed CEBPα protein expression. CEBPα is an essential transcription factor that regulates multiple aspects of myelopoiesis. Understanding how the unfolded protein response trigger down-regulation of CEBPα and lead to differentiation blockage opens new possibilities for the design of anti-AML therapeutic strategies.

      PubDate: 2016-08-10T04:18:27Z
      DOI: 10.1016/j.achaem.2016.07.001
      Issue No: Vol. 47, No. 3 (2016)
       
  • Koncentrat płytek krwi napromieniowany czy poddany redukcji biologicznych
           czynników chorobotwórczych – który składnik wybrać w celu
           profilaktyki poprzetoczeniowej choroby
           przeszczep-przeciw-gospodarzowi'
    • Authors: Piotr Olbromski; Piotr Radziwon
      Pages: 205 - 210
      Abstract: Publication date: Available online 24 April 2016
      Source:Acta Haematologica Polonica
      Author(s): Piotr Olbromski, Piotr Radziwon
      Attributable mortality due to transfusion-associated graft-versus-host disease (TA-GVHD) in contrary to the reaction occurring after transplantations has been estimated to be more than 95%. This type of reaction may occur not only in immunocompromised patients but also in patients without immune deficits. Because there is currently no available treatment for TA-GVHD and very high mortality the goal has been to reduce the likelihood of occurrence in recipients at risk of TA-GVHD. The removal of all lymphocytes from blood components is very difficult and highly impracticable. Much easier way to neutralize lymphocytes presents irreversible injury of their genetic material. It can be achieved by using ionizing irradiation (gamma- or X-ray) or pathogen reduction technologies (PRT). PRT use ultraviolet light with or without photosensitizers. Since PRT cause damage to nucleic acids, they also have potential to inactivate lymphocytes T, making them unable to proliferate, engraft and cause TA-GVHD. Transmission of infections, particularly those not routinely tested in blood components, presents still significant problem in spite of constant improvement of methods applied for donor qualification, blood testing and preparation techniques. PRT have a goal to minimize the risk of transfusion-related pathogen transmission. PRT are equally effective in TA-GVHD prophylaxis as gamma irradiation and according to current guidelines may be applied alternatively. However PRT have advantage over gamma irradiation because they additionally decrease the risk of pathogen transmission via platelet concentrate transfusion and limit proinflammatory cytokine production as well as lymphocyte activation in blood components.

      PubDate: 2016-04-26T21:30:07Z
      DOI: 10.1016/j.achaem.2016.04.004
      Issue No: Vol. 47, No. 3 (2016)
       
  • Monocyty z ekspresją Tie-2 u chorych na przewlekłą białaczkę
           limfocytową
    • Authors: Agnieszka Bojarska-Junak; Piotr Grundszok; Małgorzata Waldowska; Justyna Woś; Sylwia Chocholska; Iwona Hus; Wioleta Kowalska; Katarzyna Gęca; Waldemar Tomczak; Jacek Roliński
      Pages: 211 - 218
      Abstract: Publication date: Available online 16 August 2016
      Source:Acta Haematologica Polonica
      Author(s): Agnieszka Bojarska-Junak, Piotr Grundszok, Małgorzata Waldowska, Justyna Woś, Sylwia Chocholska, Iwona Hus, Wioleta Kowalska, Katarzyna Gęca, Waldemar Tomczak, Jacek Roliński
      Introduction In peripheral blood, monocytes form a heterogeneous population of cells. One particular subset of circulating monocytes is expressing the angiopoietin receptor Tie-2 (Tie2-expressing monocyte; TEM). TEM are characterized by tumor promoting properties. However, the role of TEM in chronic lymphocytic leukemia (CLL) immunopathogenesis remains undefined. Material and Methods Here, we evaluated the monocytes with Tie-2 expression (CD14+Tie-2+) in peripheral blood of CLL patients (n=55) and normal subjects (n=15) by flow cytometry. We investigated possible associations between TEM and poor prognostic factors such as CD38 or ZAP-70 expression, Rai stage and unfavorable cytogenetic abnormalities. Moreover, we investigated the association of TEM percentage with CD14++CD16+ monocytes and Treg percentages. Results We found that CLL patients had a higher percentage of CD14+Tie-2+ monocytes compared to normal controls. The percentage of TEM was positively associated with ZAP-70 expression as well as with unfavourable cytogenetic changes: del(17p) and/or del(11q). The frequency of TEM increased with the disease stage. We showed no correlation between the percentage of TEM and CD38 expression. The percentage of TEM at diagnosis was associated with white blood cell count as well as with the percentages of CD19+CD5+ lymphocytes and Tregs. The majority of CD14+Tie-2+ cells belonged to the intermediate monocytes subset (CD14++CD16+) while fewer of them were among the classical (CD14++CD16−) or non-classical monocyte (CD14+CD16++) subsets. TEM and CD14++CD16+ monocytes have a proangiogenic activity, suppress T-cell activation and promote Treg expansion. The results suggest that monitoring of TEM number and function may provide useful information in determining disease activity.

      PubDate: 2016-08-20T04:57:13Z
      DOI: 10.1016/j.achaem.2016.08.001
      Issue No: Vol. 47, No. 3 (2016)
       
  • Retrospektywna analiza skuteczności nilotynibu i dazatynibu w drugiej
           linii terapii przewlekłej białaczki szpikowej w polskich ośrodkach
           hematologicznych [Acta Haematologica Polonica 2016; 47(3): 219–225]
    • Authors: Anna Stępień; Krzysztof Jamroziak; Tomasz Sacha; Dorota Link-Lenczowska; Izabela Florek; Witold Prejzner; Zofia Specht-Szwoch; Małgorzata Całbecka; Marcin Rymko; Marek Dudziński; Paulina Wieszczy; Krzysztof Warzocha; Janina Góra-Tybor
      Pages: 219 - 225
      Abstract: Publication date: Available online 14 November 2016
      Source:Acta Haematologica Polonica
      Author(s): Anna Stępień, Krzysztof Jamroziak, Tomasz Sacha, Dorota Link-Lenczowska, Izabela Florek, Witold Prejzner, Zofia Specht-Szwoch, Małgorzata Całbecka, Marcin Rymko, Marek Dudziński, Paulina Wieszczy, Krzysztof Warzocha, Janina Góra-Tybor


      PubDate: 2016-11-20T09:53:14Z
      DOI: 10.1016/j.achaem.2016.08.002
      Issue No: Vol. 47, No. 3 (2016)
       
  • Retrospektywna analiza skuteczności nilotynibu i dazatynibu w drugiej
           linii terapii przewlekłej białaczki szpikowej w polskich ośrodkach
           hematologicznych
    • Authors: Anna Stępień; Krzysztof Jamroziak; Tomasz Sacha; Dorota Link-Lenczowska; Izabela Florek; Witold Prejzner; Zofia Specht-Szwoch; Małgorzata Całbecka; Marcin Rymko; Marek Dudziński; Paulina Wieszczy; Krzysztof Warzocha; Janina Góra-Tybor
      Pages: 219 - 225
      Abstract: Publication date: Available online 17 August 2016
      Source:Acta Haematologica Polonica
      Author(s): Anna Stępień, Krzysztof Jamroziak, Tomasz Sacha, Dorota Link-Lenczowska, Izabela Florek, Witold Prejzner, Zofia Specht-Szwoch, Małgorzata Całbecka, Marcin Rymko, Marek Dudziński, Paulina Wieszczy, Krzysztof Warzocha, Janina Góra-Tybor
      Until now, there has been no randomized study directly comparing the activity of second-generation BCR-ABL tyrosine kinase inhibitors (TKI-2G) nilotinib and dasatinib in chronic myeloid leukemia (CML). The aim of our study was to retrospectively analyze efficacy of nilotinib and dasatinib in the real life setting of CML with resistance or intolerance of imatinib. Of 108 included patients treated in polish hematology centers, 75 received dasatinib and 33 patients received nilotinib. Rates of complete cytogenetic response (CCyR) did not differ between the two groups of patients. After six months of therapy, CCyR was achieved in 34.7% of patients treated with dasatinib and 38.7% treated with nilotinib (p=0.86), while after 12 months, the CCyR rates were 60.0% and 77.0% in dasatinib and nilotinib groups, respectively (p=0.11). Moreover, we have not observed any significant difference in the probability of progression-free survival (p=0.89) or overall survival (p=0.99) between patients treated with these two TKI-2G. In conclusion, the results of our analysis indicate that nilotinib and dasatinib have comparable and satisfactory efficacy in the treatment of CML patients refractory or intolerant to imatinib. Our findings support current strategy of choice of IKT-2G according to drug toxicity profile and risk of specific adverse events in an individual patient.

      PubDate: 2016-08-20T04:57:13Z
      DOI: 10.1016/j.achaem.2016.08.002
      Issue No: Vol. 47, No. 3 (2016)
       
  • Effective pain reduction during bone marrow biopsy and aspiration –
           Technique over experience
    • Authors: Emil Jaddini; Nikolaj Hjortholm; Emilian Snarski
      Pages: 226 - 231
      Abstract: Publication date: Available online 20 April 2016
      Source:Acta Haematologica Polonica
      Author(s): Emil Jaddini, Nikolaj Hjortholm, Emilian Snarski
      Background: Bone marrow biopsy and aspiration (BMBA) is a diagnostic procedure within the field of internal medicine. The intensity of the pain felt by the patients often goes unrecognized by the medical staff. No extant studies have addressed pain intensity experienced during each particular step of a BMBA. Objectives: The aim was to analyze the pain intensity and explore the extent to which the technique applied by the doctor performing the biopsy influences the pain level. Methods: A survey comprising 17 questions was created and it addressed characteristics of the patients, previous experience and pain intensity on each step of the procedure. Results: 125 patients were enrolled into the study. Age (p =0.009), gender (p =0.02), pain during previous biopsies (p <0.0001) and adequate information (p =0.04) were shown to have significant impact on the pain intensity levels. There was a significant difference in the pain levels on different steps of the procedure perceived by the patients, when comparing the doctors with similar experience performing BMBA (p =0.01 to p <0.0001 depending on the step of the procedure). Conclusions: The differences in the pain scores between the doctors are most likely caused by differences in technique of BMBA. The analysis of the individual technique of the doctors performing the least painful biopsies may give answers needed for educational intervention aimed at pain reduction during BMBA.

      PubDate: 2016-04-21T17:46:37Z
      DOI: 10.1016/j.achaem.2016.04.003
      Issue No: Vol. 47, No. 3 (2016)
       
  • Zalecenia Polskiej Grupy Szpiczakowej dotyczące rozpoznawania i leczenia
           
    • Authors: Anna Dmoszyńska; Adam Walter-Croneck; Barbara Pieńkowska-Grela; Lidia Usnarska-Zubkiewicz; Jan Walewski; Grzegorz Charliński; Wiesław Wiktor Jędrzejczak; Elżbieta Wiater; Ewa Lech-Marańda; Krzysztof Jamroziak; Agnieszka Druzd-Sitek; Dominik Dytfeld; Mieczysław Komarnicki; Tadeusz Robak; Artur Jurczyszyn; Joanna Mańko; Aleksander Skotnicki; Sebastian Giebel; Ryszard Czepko; Janusz Meder; Bogdan Małkowski; Krzysztof Giannopoulos
      Pages: 39 - 85
      Abstract: Publication date: Available online 30 April 2016
      Source:Acta Haematologica Polonica
      Author(s): Anna Dmoszyńska, Adam Walter-Croneck, Barbara Pieńkowska-Grela, Lidia Usnarska-Zubkiewicz, Jan Walewski, Grzegorz Charliński, Wiesław Wiktor Jędrzejczak, Elżbieta Wiater, Ewa Lech-Marańda, Krzysztof Jamroziak, Agnieszka Druzd-Sitek, Dominik Dytfeld, Mieczysław Komarnicki, Tadeusz Robak, Artur Jurczyszyn, Joanna Mańko, Aleksander Skotnicki, Sebastian Giebel, Ryszard Czepko, Janusz Meder, Bogdan Małkowski, Krzysztof Giannopoulos
      New drugs introduced in recent years for the therapy of multiple myeloma patients resulted in better responses and prolongation of overall survival. While therapeutic regimens based on bortezomib and thalidomide are recommended to most patients in first-line therapy, lenalidomide represents a cornerstone for treatment of relapsed/refractory myeloma patients. Most patients profit from prolonged treatment composed of consolidation and maintenance or treatment till progression. Beside concept of longer treatment, it is recommended to start therapy in some patients earlier, taking into consideration biomarkers of active disease. In this article, we described also therapeutic recommendation for Waldenström macroglobulinemia and other plasmocytic dyscrasias.

      PubDate: 2016-05-01T23:00:18Z
      DOI: 10.1016/j.achaem.2016.04.010
      Issue No: Vol. 47, No. 2 (2016)
       
  • Część I: Wytyczne postępowania w hemofilii A i B niepowikłanej
           inhibitorem czynnika VIII i IX (wydanie zaktualizowane)
    • Authors: Jerzy Windyga; Krzysztof Chojnowski; Anna Klukowska; Magdalena Łętowska; Andrzej Mital; Jacek Musiał; Jarosław Peregud-Pogorzelski; Maria Podolak-Dawidziak; Jacek Treliński; Anetta Undas; Tomasz Urasiński; Joanna Zdziarska; Krystyna Zawilska
      Pages: 86 - 114
      Abstract: Publication date: Available online 28 April 2016
      Source:Acta Haematologica Polonica
      Author(s): Jerzy Windyga, Krzysztof Chojnowski, Anna Klukowska, Magdalena Łętowska, Andrzej Mital, Jacek Musiał, Jarosław Peregud-Pogorzelski, Maria Podolak-Dawidziak, Jacek Treliński, Anetta Undas, Tomasz Urasiński, Joanna Zdziarska, Krystyna Zawilska
      In this document, Working Group for Haemostasis of the Polish Society of Haematology and Blood Transfusion updates the principles of diagnosis and treatment of haemophilia A and B, published for the first time in 2008. In a companion paper (part II), the management of haemophilia A and B complicated by factor VIII and IX inhibitors will be covered.

      PubDate: 2016-05-01T23:00:18Z
      DOI: 10.1016/j.achaem.2016.04.009
      Issue No: Vol. 47, No. 2 (2016)
       
  • Zmiany rodzaju koncentratu czynnika krzepnięcia u chorego na hemofilię
           – korzyść czy ryzyko?
    • Authors: Anna Klukowska
      Pages: 115 - 120
      Abstract: Publication date: Available online 19 March 2016
      Source:Acta Haematologica Polonica
      Author(s): Anna Klukowska
      Patients with haemophilia are treated with a variety of coagulation factor concentrates. They usually change concentrate types many times during their lifetime for different, often independent, reasons. Since recombinant coagulation factor concentrates VIII and IX became available, some patients in well-developed countries have started using these products instead of previously applied plasma-derived concentrates. At the moment, there are different types of concentrates available, including first, second and third generation recombinant concentrates, and factor VIII concentrates with full length or B deleted domain molecule. The drug's efficacy and safety are both taken into consideration when evaluating coagulation concentrates. When we talk of concentrates’ safety, we consider virus safety, non-increased risk of inhibitor development and no adverse events as a potential threat to the patient's health. With the modern methods of blood donor tests and effective methods of viral inactivation having been introduced, the plasma-derived coagulation factor concentrates are currently as safe as recombinant concentrates with regard to elimination of enveloped viruses, including HIV or HCV. However, they still fail to eliminate completely the transmission of non-enveloped viruses such as e.g. hepatitis A virus (HAV) or parvovirus B19. Meanwhile, the risk of inhibitor development continues to be the subject of investigation. The results of many studies indicate there are no statistically significant differences in inhibitor incidence between patients treated with plasma-derived coagulation factor concentrates and recombinant concentrates but the results of randomized study (SIPPET) presented in December 2015 during ASH show 1,87-fold higher inhibitor incidence in recombinant factor VIII group than the plasma derived group in previous untreated children. The question concerning the potentially increased risk of inhibitor development following a switch from plasma-derived to recombinant coagulation factor concentrates, or a switch from one recombinant concentrate to another in previously treated patients, should thus be answered negatively. At the same time, further investigation is required in this field, particularly in small children.

      PubDate: 2016-03-20T22:40:39Z
      DOI: 10.1016/j.achaem.2016.03.001
      Issue No: Vol. 47, No. 2 (2016)
       
  • Nowe metody diagnostyczne w prognozowaniu i śledzeniu odpowiedzi na
           leczenie inhibitorami kinaz tyrozynowych przewlekłej białaczki szpikowej
           
    • Authors: Joanna Wącław; Tomasz Sacha
      Pages: 121 - 127
      Abstract: Publication date: Available online 28 April 2016
      Source:Acta Haematologica Polonica
      Author(s): Joanna Wącław, Tomasz Sacha
      Techniques of molecular biology are of key importance in diagnostics and monitoring of tyrosine kinase inhibitors (TKIs) treatment response of chronic myeloid leukemia (CML). Although much has already been achieved in this field, new prognostic factors and developments in laboratory methods are constantly emerging. Halving time is a new prognostic factor defined as the rate of BCR-ABL1 decline from baseline, assessed by estimating the number of days over which BCR-ABL1 halved. Among patients with >10% BCR-ABL1 at 3 months of therapy with TKI, the poorest-risk group can be distinguished by the slow rate of BCR-ABL1 decline from baseline. More common use of techniques, such as the whole exome sequencing and transcriptome sequencing will enable assessment of patients’ genetic variation at diagnosis and may contribute to a prognostic score that will allow for optimization of therapy. Digital PCR built on traditional PCR provides highly sensitive absolute quantification of BCR-ABL1 transcript level without the need for standard curves and could further improve the treatment results in patients with CML.

      PubDate: 2016-05-01T23:00:18Z
      DOI: 10.1016/j.achaem.2016.04.007
      Issue No: Vol. 47, No. 2 (2016)
       
  • Ponatynib w leczeniu przewlekłej białaczki szpikowej i ostrej białaczki
           limfoblastycznej z chromosomem Filadelfia
    • Authors: Tomasz Sacha
      Pages: 128 - 138
      Abstract: Publication date: Available online 22 April 2016
      Source:Acta Haematologica Polonica
      Author(s): Tomasz Sacha
      Ponatinib is a third generation tyrosine kinase inhibitor (TKI) active against wild type and mutant bcr/abl tyrosine kinase (including T315I). Ponatinib is approved for the treatment of adult patients with T315I mutation detected in CML (all phases) and in Ph+ ALL, for CML patients who are resistant to dasatinib or nilotinib and patients with Ph+ ALL who are intolerant or resistant to dasatinib and for whom subsequent imatinib treatment is not an optimal therapy. In phase II registration study (PACE), 267 patients with CML in chronic phase (CP), 83 in accelerated (AC) and 94 patients in blastic phase (BP) of CML or Ph+ALL were enrolled. T315I mutation was detected in 64, 18 and 46 patients, respectively. Two or more or three or more TKIs were used before ponatinib in 93% and 58% of patients, respectively for median period of 16.7 months. Major cytogenetic response (MCyR) by 12 months was achieved in 59% of CP CML patients (median follow-up 36 months), and major hematological response (MaHR) by 6 months in 55% of AC CML patients, and in 34% of BP CML and Ph+ALL patients (median follow-up 15.16 and 6 months, respectively). Efficacy of ponatinib as a first-line treatment for CP CML patients was shown in phase II and III trials. Serious adverse reactions have been reported with ponatinib, including vascular occlusion, heart failure and hepatotoxicity. Ponatinib is a valuable treatment option for patients with CML and Ph+ALL resistant or intolerant to previous therapy with TKIs and those with T315I mutation.

      PubDate: 2016-04-26T21:30:07Z
      DOI: 10.1016/j.achaem.2016.04.002
      Issue No: Vol. 47, No. 2 (2016)
       
  • Metformina: stary lek w nowej aplikacji
    • Authors: Anna Dmoszyńska; Monika Podhorecka; Krzysztof Giannopoulos
      Pages: 139 - 145
      Abstract: Publication date: Available online 20 April 2016
      Source:Acta Haematologica Polonica
      Author(s): Anna Dmoszyńska, Monika Podhorecka, Krzysztof Giannopoulos
      Metformin is a biguanide derivative that was synthesized in the 20thies years of last century. The main mechanism of action is the ability to inhibit hepatic gluconeogenesis and trigger glucose uptake in skeletal muscles. Metformin is not only an antihyperglycaemic drug but also has protective effects on endothelium cells. In the last decade, many publications displayed that metformin can reduce risk of cancer in diabetic patients and demonstrated a protective effect against certain types of cancer. In this review article, we discuss known mechanisms of metformin anticancer action and its beneficial role as vascular protective and anticancer agent.

      PubDate: 2016-04-21T17:46:37Z
      DOI: 10.1016/j.achaem.2016.04.001
      Issue No: Vol. 47, No. 2 (2016)
       
  • Rzadkie choroby makrofagów u dorosłych
    • Authors: Maciej Machaczka; Monika Klimkowska
      Pages: 146 - 154
      Abstract: Publication date: Available online 28 April 2016
      Source:Acta Haematologica Polonica
      Author(s): Maciej Machaczka, Monika Klimkowska
      Diseases of macrophages are rare entities characterized by the accumulation of macrophages, dendritic cells or monocyte-derived cells in various tissues and organs. This article focuses on macrophage disorders which can be present in adult patients. The review highlights pathogenesis, signs and symptoms, diagnostic criteria and principles of therapy in the most frequent macrophage diseases in adults: hemophagocytic lymphohistiocytosis, Rosai-Dorfman disease, Gaucher disease, Niemann-Pick disease, and Langerhans cell histiocytosis. Since macrophage disorders can be encountered by various medical specialists, basic knowledge of these entities and their diagnostic criteria should be familiar to all physicians, including hematologists.

      PubDate: 2016-05-01T23:00:18Z
      DOI: 10.1016/j.achaem.2016.04.006
      Issue No: Vol. 47, No. 2 (2016)
       
  • Inhibitory immunologicznych punktów kontrolnych podziału komórki w
           leczeniu chorób nowotworowych
    • Authors: Aleksandra Mędra; Agata Majchrzak; Piotr Smolewski
      Pages: 155 - 162
      Abstract: Publication date: Available online 25 April 2016
      Source:Acta Haematologica Polonica
      Author(s): Aleksandra Mędra, Agata Majchrzak, Piotr Smolewski
      Despite of great progress in anti-neoplastic treatment several solid tumors and hematologic malignancies still remain incurable. Immune system remains under control of several controlling mechanisms. Genetic or epigenetic changes in neoplastic cells provide antigen-derived diversity; however, these cells do not initiate immune response. The main mechanism of development of immune resistance by tumor cells seems to be a change in expression of proteins engaged in the immune control point. Immunotherapy with immune checkpoint inhibitors has emerged as promising modality of tumors showing response to several antigens, e.g. anti-CTLA-4 or PD1-PDL1 monoclonal antibodies. In this review we demonstrate the state in the field on this modality of anti-neoplastic treatment.

      PubDate: 2016-04-26T21:30:07Z
      DOI: 10.1016/j.achaem.2016.04.008
      Issue No: Vol. 47, No. 2 (2016)
       
  • Interferon γ i interleukina-2 a ostra choroba
           przeszczep-przeciw-gospodarzowi i powikłania infekcyjne u pacjentów po
           allogenicznej transplantacji komórek krwiotwórczych
    • Authors: Malwina Rybicka; Mirosław Markiewicz; Elżbieta Pietruszka; Karol Goraus; Aleksandra Suszka-Świtek; Ryszard Wiaderkiewicz; Sylwia Mizia; Monika Dzierżak-Mietła; Krzysztof Białas; Sławomira Kyrcz-Krzemień
      Pages: 163 - 168
      Abstract: Publication date: Available online 25 April 2016
      Source:Acta Haematologica Polonica
      Author(s): Malwina Rybicka, Mirosław Markiewicz, Elżbieta Pietruszka, Karol Goraus, Aleksandra Suszka-Świtek, Ryszard Wiaderkiewicz, Sylwia Mizia, Monika Dzierżak-Mietła, Krzysztof Białas, Sławomira Kyrcz-Krzemień
      Introduction The allogeneic Hematopoietic Stem Cells Transplantation (alloHSCT) is associated with the risk of Graft versus Host Disease (GvHD) and infections. The pathogenesis of acute GvHD is related to T-lymphocytes, which identify alloantigens on host's Antigen Presenting Cells, induce production of IFN-γ and IL-2, recruit the immunological effectory cells and destroy tissues and organs. Aim The aim of the study was to analyse the relationship between IL-2 and IFN-γ serum concentrations and acute GvHD and infections. Material and methods The study involved 62 patients, 30 (48%) male and 32 (52%) female, aged at median 49.5 (19–68) years, after alloHSCT from sibling (n=12) or from unrelated donor (n=50) performed for acute myeloid leukemia (AML) with myeloablative conditioning (n=26, 42%) and with non-myeloablative conditioning (n=36, 58%) in Katowice in years 2012–2014. All patients received standard immunosuppressive therapy with cyclosporin-A and methotrexate plus pre-transplant anti-thymocyte globulin in unrelated setting. Blood samples were collected pre-transplant before start and after (on day -1) the conditioning therapy and on days +2 +4, +6, +10, +20, +30 after alloHSCT. The IL-2 and IFN-γ serum concentrations were determined with use of ELISA assay. Before statistical analysis patients were divided into 4 groups according to the presence of acute GvHD and clinical manifestation of bacterial, viral or fungal infection. Results Group I – patients with neither acute GvHD nor infectious complications, n=15 (24%), group II – patients with infectious complications without acute GvHD, n=17 (27%), group III – patients with acute GvHD without infectious complications, n=9 (15%), and group IV – patients with both acute GvHD and infectious complications, n=21 (34%). IFN-γ levels were higher in group II than in other groups on days +20 (p=0.014) and +30 (p=0.008). The POST-HOC tests revealed lower levels of IFN-γ on day +30 in group I (p=0.039) and in group IV (p=0.017) as compared to group II. The concentration of IL-2 was undetectable in almost all patients at all studied time points. Conclusion Serum concentration of IFN-γ following alloHSCT gradually increases. High serum concentration of IFN-γ is related to infectious complications rather than to acute GvHD. Undetectable serum concentration of IL-2 in majority of patients prevents from drawing conclusions.

      PubDate: 2016-04-26T21:30:07Z
      DOI: 10.1016/j.achaem.2016.04.005
      Issue No: Vol. 47, No. 2 (2016)
       
  • Zmiany białaczkowe w ośrodkowym układzie nerwowym w przebiegu
           przewlekłej białaczki limfocytowej
    • Authors: Ewa Wąsik-Szczepanek; Dariusz Szczepanek; Małgorzata Wach; Marek Hus
      Pages: 10 - 14
      Abstract: Publication date: Available online 2 February 2016
      Source:Acta Haematologica Polonica
      Author(s): Ewa Wąsik-Szczepanek, Dariusz Szczepanek, Małgorzata Wach, Marek Hus
      Central nervous system involvement (CNS) of chronic lymphocytic leukemia (CLL) is a rare complication. Some analyses of autopsy studies suggest that the complication remains subclinical or is under-diagnosed. The symptoms of patients with leukemic CNS involvement are heterogeneous and nonspecific. Due to the low incidence, there are no treatment guidelines for this problem. Intrathecal chemotherapy and cranial irradiation are often performed.

      PubDate: 2016-02-27T21:04:56Z
      DOI: 10.1016/j.achaem.2016.01.002
      Issue No: Vol. 47, No. 1 (2016)
       
  • Elevated factor VIII activity and venous thromboembolism in patients
           referred to the Iranian Blood Transfusion Organization: A case control
           study
    • Authors: Seyed Mohammad Reza Tabatabaei; Seyed Mehdi Sajjadi; Minoo Ahmadi Nejad; Farzaneh Tavasoli; Azita Azarkeivan
      Pages: 15 - 18
      Abstract: Publication date: Available online 2 February 2016
      Source:Acta Haematologica Polonica
      Author(s): Seyed Mohammad Reza Tabatabaei, Seyed Mehdi Sajjadi, Minoo Ahmadi Nejad, Farzaneh Tavasoli, Azita Azarkeivan
      Objective A high plasma level of factor eight (FVIII) is a risk factor for venous thromboembolism (VTE). Since, there was no report about the association of elevated FVIII and VTE in Iranian population, the incidence of elevated FVIII and its association to VTE was evaluated. Materials and methods 152 consecutive idiopathic VTE patients referred to the Iranian Blood Transfusion Organization (IBTO) and 130 healthy matched blood donors were studied. At least one confirmed idiopathic deep vein thrombosis (DVT) or pulmonary embolism (PE) was found among all cases. The blood samples were collected at least 3 months after DVT/PE diagnosis. The normal reference range was determined by using the Control samples of the donors. FVIII levels were measured using PTT based one-staged assay. Results The FVIII levels in the cases and controls were 157.3±53.4 and 111.8±29.7, respectively. In cases, the lowest and the highest levels of FVIII were 66IU/dl and 364IU/dl, while they were 42IU/dl and 195IU/dl for the controls. There was no relation between gender, age and FVIII level in either group. The normal reference range for the controls was 52–171IU/dl. Considering the cut-off point as 180IU/dl, the elevated values were seen in 28.9% of the case group vs. 3.1% of the control group. Conclusion Elevated factor VIII is likely to be a risk factor for VTE. Moreover, a new normal reference range for the Iranian population was defined.

      PubDate: 2016-02-27T21:04:56Z
      DOI: 10.1016/j.achaem.2016.01.001
      Issue No: Vol. 47, No. 1 (2016)
       
  • The patient with 5q minus syndrome and JAK2 V617F mutation with the
           presence of ringed erythroblasts meeting the criteria of RARS-T
           effectively treated with lenalidomide – A case report
    • Authors: Marcin Kruszewski; Adriana Czyż; Krzysztof Lewandowski; Monika Prochorec-Sobieszek; Jarosław Czyż
      Pages: 29 - 32
      Abstract: Publication date: Available online 7 January 2016
      Source:Acta Haematologica Polonica
      Author(s): Marcin Kruszewski, Adriana Czyż, Krzysztof Lewandowski, Monika Prochorec-Sobieszek, Jarosław Czyż
      5q minus syndrome is a form of myelodysplastic syndrome characterized by the presence of an isolated deletion of long arm of the chromosome 5. Patients with 5q minus respond well to the treatment with lenalidomide. The presence of the JAK2 V617F mutation is a common feature of refractory anemia with ring sideroblasts and marked thrombocytosis. Much less is known about effectiveness of lenalidomide in these patients. We present the patient with 5q minus syndrome and JAK2 V617F mutation accompanied by the presence of ringed erythroblasts meeting the criteria of RARS-T. We could identify only two such patients reported in the literature; no details were given about effectiveness of lenalidomide in that group. We observed good response to the treatment with lenalidomide with transfusion independence 9 months after starting of the treatment; however, there was no complete eradication of del (5)(q13q31) clone nor the clone with JAK V617F mutation.

      PubDate: 2016-01-10T09:56:40Z
      DOI: 10.1016/j.achaem.2015.12.002
      Issue No: Vol. 47, No. 1 (2016)
       
  • Limfohistiocytoza hemofagocytarna związana z infekcją –
           opis przypadku
    • Authors: Karolina Torba; Grzegorz Helbig; Krzysztof Woźniczka; Anna Wacławik; Sławomira Kyrcz-Krzemień
      Pages: 33 - 37
      Abstract: Publication date: Available online 19 February 2016
      Source:Acta Haematologica Polonica
      Author(s): Karolina Torba, Grzegorz Helbig, Krzysztof Woźniczka, Anna Wacławik, Sławomira Kyrcz-Krzemień
      Hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially fatal disorder characterized by abnormal activation of macrophages. It is also characterized by hemophagocytosis in the bone marrow and in the reticuloendothelial system (RES). The most common symptoms are persistent fever, splenomegaly and cytopenia. The probable mechanism of disease is due to hyperinflammation caused by increasing amounts of proinflammatory cytokines. As a consequence numerous metabolic disturbances with multiple organ failure occur. Without a proper treatment this disease may have a fatal outcome. Herein we present a 24-year-old male with HLH who achieved a rapid response to the therapy despite the initial poor overall condition which was associated with an advanced disease stage as well as prolonged diagnostic process.

      PubDate: 2016-02-27T21:04:56Z
      DOI: 10.1016/j.achaem.2016.02.001
      Issue No: Vol. 47, No. 1 (2016)
       
  • Genetic alterations in B-acute lymphoblastic leukemia
    • Authors: Mohammadi Seyedeh Momeneh; Mohammad Nejad Daryosh; Nozad Charoudeh Hojjatollah
      Abstract: Publication date: Available online 6 December 2016
      Source:Acta Haematologica Polonica
      Author(s): Mohammadi Seyedeh Momeneh, Mohammad Nejad Daryosh, Nozad Charoudeh Hojjatollah
      Considerable advances have been made in treatment of acute lymphoblastic leukemia (ALL) with an overall survival rate of 85% in children, and with a great improvement in adults. Despite this improvements and the accessibility of hematopoietic stem cell transplantation, relapsed ALL remains a leading cause of childhood mortality emphasizing the need of new approaches on therapy. Understanding of the pathobiology and genetic alteration of ALL has been enhanced by developing molecular technologies including microarray analysis and genome sequencing. These studies have helped identifying mutations in key signaling pathways and revolutionized the treatment of ALL by drugs which specifically target the genetic defects of leukemia cells, such as tyrosine kinase inhibitors. In this paper, we review the clinically important Genetic Alterations in ALL.

      PubDate: 2016-12-11T09:29:15Z
      DOI: 10.1016/j.achaem.2016.11.002
       
  • Emerging spread of β-thalassaemia trait in Nigeria
    • Authors: Akanni E. Olufemi; Bamisaye E. Oluwaseyi; Alabi T. Temitope
      Abstract: Publication date: Available online 6 December 2016
      Source:Acta Haematologica Polonica
      Author(s): Akanni E. Olufemi, Bamisaye E. Oluwaseyi, Alabi T. Temitope
      Background Chronic anaemia mainly thalassaemia and sickle cell anaemia are inherited disorders of haemoglobin. Presently about 7% of the world's populations are carriers of a potentially pathological haemoglobin gene. Sickle cell disease is a common haemoglobinopathy in Nigeria but recently cases of β-thalassaemia traits are becoming prominent. This study aimed at screening for β-thalassaemia in adults and children with chronic anaemia in Nigeria by assessing the patients’ level of haemoglobin F, haemoglobin A2 and red cell indices. Materials and methods Haemoglobin F and HbA2 were determined in the chronic anaemia patients by Alkaline Denaturation Method and Beta-Thal HbA2 Quick Column Procedure respectively. Haemoglobin genotype was determined by Haemoglobin Electrophoresis at alkaline medium while Complete Blood count was estimated using Sysmex KX-2IN Autoanalyser. Results The mean HbF, HbA2, HCT, MCV, MCH and MCHC of the children and adults are 2.56±0.46 and 2.45±0.87; 2.05±0.25 and 1.89±0.60; (20.96±3.56) and (21.15±3.12); (78.69±14.11) and (81.58±12.59); (23.07±7.36) (22.74±5.39); (31.23±14.32) and (27.52±3.84) respectively. Four percent (2 subjects) of each adult and children population had increased HbF level (>1.5%) and HbA2 levels (>2.8%) and these subjects are composed of 2 children with haemoglobin genotype AA and two adult with haemoglobin genotypes SS. Conclusions The outcome of this study reiterates the emergence of β-thalassaemia traits and iron deficiency anaemia in different parts of Nigeria irrespective of their haemoglobin genotype status. This requires adequate specialized intervention for their diagnosis and treatment. There is therefore the need for subsequent molecular analysis to determine the β-thalassaemia genes present in the studied community.

      PubDate: 2016-12-11T09:29:15Z
      DOI: 10.1016/j.achaem.2016.11.003
       
  • Single Positive Commensal Blood Culture in hospital setting is associated
           with higher mortality after hematopoietic stem cell transplantation
    • Authors: Krzysztof Bogusz; Emilian Snarski; Patrycja Rusicka; Kazimierz Hałaburda; Tigran Torosian; Małgorzata Rokicka; Grzegorz Basak; Monika Paluszewska; Piotr Boguradzki; Grzegorz Charliński; Magdalena Tormanowska; Wiesław Wiktor Jędrzejczak
      Abstract: Publication date: Available online 6 December 2016
      Source:Acta Haematologica Polonica
      Author(s): Krzysztof Bogusz, Emilian Snarski, Patrycja Rusicka, Kazimierz Hałaburda, Tigran Torosian, Małgorzata Rokicka, Grzegorz Basak, Monika Paluszewska, Piotr Boguradzki, Grzegorz Charliński, Magdalena Tormanowska, Wiesław Wiktor Jędrzejczak
      Background Single positive staphylococcal blood culture in a hematopoietic stem cell transplantation (HSCT) recipient is generally regarded as contamination. Such a blood culture (BC) does not fill the criteria for Laboratory-Confirmed Bloodstream Infection (LCBI) and could be described as Single Positive Commensal Blood Culture. The aim of this retrospective cohort analysis was to determine the clinical significance of SPCBC in HSCT recipients. Methods 206 patients transplanted between 2007 and 2013 were followed until January 2015. Results The 100-day survival for patients without positive BC was 99.6% compared with 83.9% for LCBI and 82.8% for SPCBC (p =0.0036). The 5-year overall survival (5yOS) was 67.1% for patients without positive BC, 44.9% for LCBI, 34.0% for SPCBC (p <0.0001). The per-day risk of developing SPCBC was identical in autologous and allogenic transplantation. SPCBC remained a significant factor for reduced 5yOS after HSCT in the univariate analysis (HR 2.52, 1.26–5.02, p =0.0001) as well as in the multivariate analysis (HR 2.21, 1.26–3.87, p =0.006). SPCBC consisted solely of different Staphylococcus species with dominance of Staphylococcus epidermidis (64% of SPCBC). Conclusion To our knowledge this is the first report that specifically shows that short- and long-term survival after HSCT is significantly lower in patients who experience an episode of SPCBC with Staphylococcus spp. during HSCT hospitalization.

      PubDate: 2016-12-11T09:29:15Z
      DOI: 10.1016/j.achaem.2016.11.004
       
  • Transfusion-associated graft-vs-host disease – A case report
    • Authors: Jina Bhattacharyya; Nilom Khound; Bhaskar Jyoti Kakati; Mrinal Bhattacharyya; Sangit Dutta; Aishwarya Raj
      Abstract: Publication date: Available online 27 October 2016
      Source:Acta Haematologica Polonica
      Author(s): Jina Bhattacharyya, Nilom Khound, Bhaskar Jyoti Kakati, Mrinal Bhattacharyya, Sangit Dutta, Aishwarya Raj
      Transfusion-associated graft-vs-host disease (TAGVHD) is a rare complication of blood transfusion. Unlike GVHD associated with hematopoietic stem cell transplantation TA-GVHD involves the patient's bone marrow and leads to bone marrow aplasia. We report a case of TA-GVHD in a 45-year-old post-hysterectomy patient after packed red blood cell transfusion from a sibling donor. The patient had fever, maculopapular rashes all over the body, elevated transaminases, and hyperglycemia after a week of the blood transfusion. Severe pancytopenia and bone marrow aplasia followed and she succumbed to her disease after 3 weeks of onset.

      PubDate: 2016-10-30T05:10:04Z
      DOI: 10.1016/j.achaem.2016.10.005
       
  • Association of rs1319868, rs1567811 and rs8041224 of IGF1R gene with
           infection among sickle cell anemia Tunisian patients
    • Authors: Mouna Ben; Sassi Leila Chaouch Miniar Kalai Imen Moumni Houyem
      Abstract: Publication date: Available online 14 October 2016
      Source:Acta Haematologica Polonica
      Author(s): Mouna Ben Sassi, Leila Chaouch, Miniar Kalai, Imen Moumni, Houyem Ouragini, Imen Darragi, Dorra Chaouachi, Imen Boudrigua, Raouf Hafsia, Salem Abbes
      Background and aim Sickle cell anemia (SCA) is characterized by variable patterns of clinical expression. Polymorphisms linked to different genes have been associated with specific complications of the disease. Herein, we focused on the study of the association of 4 polymorphisms of Insulin like Growth Factor 1 receptor (IGF1R) gene with infections, which are the major cause of death in SCA. Material and methods This study involved 116 sickle cell patients among whom 58 SS have the same confirmed infectious phenotype. Allele-Specific PCR was performed for the study of rs1319868, whereas the PCR/sequencing method was carried out for rs1567811, rs2872060 and rs8041224. Results The results showed that rs1319868 and rs1567811 were associated with a decreased risk of infection among SS patients (p =0.038, RR=0.54; p =0.044, RR=0.56, respectively). Interestingly, the combination of different genotypes showed the association of the genotype GT of rs1319868 and the genotype CC of rs8041224 with further decreased infection risk in SCA (p =0.028, RR=0.04). Conclusion These significant associations of IGF1R SNPs with infection suggest that this gene could play an important role in the immune function in SCA.

      PubDate: 2016-10-16T03:40:05Z
       
  • Analiza mutacji talasemii alfa u chorych diagnozowanych w Instytucie
           Hematologii i Transfuzjologii
    • Authors: Edyta Klimczak-Jajor; Joanna Skulimowska Turowski Hanna Pyl Uhrynowska Katarzyna Guz
      Abstract: Publication date: Available online 14 October 2016
      Source:Acta Haematologica Polonica
      Author(s): Edyta Klimczak-Jajor, Joanna Skulimowska, Paweł Turowski, Hanna Pyl, Małgorzata Uhrynowska, Katarzyna Guz, Ewa Mendek-Czajkowska, Anna Ejduk, Izabella Kopeć, Marzena Dębska, Ewa Brojer
      Background Alpha-thalassemia is genetically transmitted hemolytic anemia resulting from disturbance of the globins chain synthesis. Alpha-thalassemia is caused most frequently by deletions and less commonly by nondeletional defects. Aim To introduce the molecular methods for routine identifications of alpha-thalassemia mutations and to study the characteristics of these mutations in Poland. Methods Blood sample of 155 patients with normal or reduced HbA2 values was obtained for blood counting. All samples underwent gap-PCR to screen for the seven common α-thal deletions and MLPA analysis. The DNA of 21 patients in which deletions were not detected has been directly sequenced. Results We detected mutations in the alpha-globin gene in 72 of 155 patients studied. 55 out of 72 cases showed most common thalassemia deletions, as the following: a single gene deletion (α3.7 and α4.2) and both genes deletion (FIL, SEA, MED I, and α20.5). Owing to the use of MLPA technique, we found nontypical deletions in another 12 patients and multiplication of the alpha-globin genes in further 4 cases. We also identified a patient with a point mutation HBA2: c.300 + 2T> A by DNA sequencing. Conclusions Molecular analysis of the alpha-globin cluster is required for a correct diagnosis in patients with normal or reduced level of HbA2. The results of the study show that due to the progressive migration of the population and globalization, thalassemia must be included in the differential diagnosis of anemia in Poland.

      PubDate: 2016-10-16T03:40:05Z
       
  • Precursor B-lymphoblastic lymphoma mimicking: An acute subdural hematoma
    • Authors: Ant Uzay; Siret Ratip Ilhan Elmaci Metin Ozdemirli
      Abstract: Publication date: Available online 14 October 2016
      Source:Acta Haematologica Polonica
      Author(s): Ant Uzay, Siret Ratip, Ilhan Elmaci, Metin Ozdemirli
      Objective and importance We present the first case of a precursor acute subdural B-lymphoblastic lymphoma mimicking an acute subdural hematoma. Clinical presentation A 19 year old male presented with an acute onset of headache, nausea and vomiting. CT scan showed crescentic right-sided, frontoparietal subdural mass isointense with cortex and showing homogeneous enhancement after gadolinium. Intervention The patient underwent a craniotomy and a gray subdural tumor with invasion of both dura and brain was observed. The invaded dura was resected and duraplasty performed. Histopathologically, the tumor was composed of small round cells infiltrating soft tissue. In some areas of the tumor, cells were arranged in a linear, “Indian file” fashion between collagen bundles. Their nuclei were generally uniform, round to ovoid in shape, small to medium in size, and featured delicate chromatin. Accompanying cytoplasm was scant. Necrosis was absent. On immunohistochemical analysis, the tumor cells were positive for CD79a, TdT, CD10 and CD34. Conclusion Subdural lymphoma can present as a neurosurgical emergency, and lymphoma should be considered as a rare but possible diagnosis before operation.

      PubDate: 2016-10-16T03:40:05Z
       
  • Limfohistiocytoza hemofagocytarna u dzieci
    • Authors: Magdalena Iwona; Malinowska
      Abstract: Publication date: Available online 13 October 2016
      Source:Acta Haematologica Polonica
      Author(s): Magdalena Wołowiec, Iwona Malinowska
      Hemophagocytic lymphohistiocytosis (HLH) is a heterogenic syndrome characterized by an acute, life-threatening inflammation due to a highly stimulated but ineffective immune response. Depending on the etiology, HLH is divided into primary (genetic) and secondary (acquired) forms. Primary HLH can be divided into familial HLH and HLH associated with other genetic disorders. Secondary HLH usually occurs in the context of a severe infection, rheumatic disease, or malignancy. HLH in children is a rare condition characterized by nonspecific symptoms and poor prognosis. Novel diagnostic tools and therapeutic methods give hope to improve the survival of the patients.

      PubDate: 2016-10-16T03:40:05Z
       
 
 
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