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Publisher: Elsevier   (Total: 3160 journals)

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Showing 1 - 200 of 3160 Journals sorted alphabetically
Academic Pediatrics     Hybrid Journal   (Followers: 37, SJR: 1.655, CiteScore: 2)
Academic Radiology     Hybrid Journal   (Followers: 25, SJR: 1.015, CiteScore: 2)
Accident Analysis & Prevention     Partially Free   (Followers: 97, SJR: 1.462, CiteScore: 3)
Accounting Forum     Hybrid Journal   (Followers: 28, SJR: 0.932, CiteScore: 2)
Accounting, Organizations and Society     Hybrid Journal   (Followers: 37, SJR: 1.771, CiteScore: 3)
Achievements in the Life Sciences     Open Access   (Followers: 5)
Acta Anaesthesiologica Taiwanica     Open Access   (Followers: 7)
Acta Astronautica     Hybrid Journal   (Followers: 428, SJR: 0.758, CiteScore: 2)
Acta Automatica Sinica     Full-text available via subscription   (Followers: 2)
Acta Biomaterialia     Hybrid Journal   (Followers: 28, SJR: 1.967, CiteScore: 7)
Acta Colombiana de Cuidado Intensivo     Full-text available via subscription   (Followers: 3)
Acta de Investigación Psicológica     Open Access   (Followers: 3)
Acta Ecologica Sinica     Open Access   (Followers: 10, SJR: 0.18, CiteScore: 1)
Acta Haematologica Polonica     Free   (Followers: 1, SJR: 0.128, CiteScore: 0)
Acta Histochemica     Hybrid Journal   (Followers: 3, SJR: 0.661, CiteScore: 2)
Acta Materialia     Hybrid Journal   (Followers: 289, SJR: 3.263, CiteScore: 6)
Acta Mathematica Scientia     Full-text available via subscription   (Followers: 6, SJR: 0.504, CiteScore: 1)
Acta Mechanica Solida Sinica     Full-text available via subscription   (Followers: 9, SJR: 0.542, CiteScore: 1)
Acta Oecologica     Hybrid Journal   (Followers: 12, SJR: 0.834, CiteScore: 2)
Acta Otorrinolaringologica (English Edition)     Full-text available via subscription  
Acta Otorrinolaringológica Española     Full-text available via subscription   (Followers: 2, SJR: 0.307, CiteScore: 0)
Acta Pharmaceutica Sinica B     Open Access   (Followers: 1, SJR: 1.793, CiteScore: 6)
Acta Poética     Open Access   (Followers: 4, SJR: 0.101, CiteScore: 0)
Acta Psychologica     Hybrid Journal   (Followers: 27, SJR: 1.331, CiteScore: 2)
Acta Sociológica     Open Access   (Followers: 1)
Acta Tropica     Hybrid Journal   (Followers: 6, SJR: 1.052, CiteScore: 2)
Acta Urológica Portuguesa     Open Access  
Actas Dermo-Sifiliograficas     Full-text available via subscription   (Followers: 3, SJR: 0.374, CiteScore: 1)
Actas Dermo-Sifiliográficas (English Edition)     Full-text available via subscription   (Followers: 2)
Actas Urológicas Españolas     Full-text available via subscription   (Followers: 3, SJR: 0.344, CiteScore: 1)
Actas Urológicas Españolas (English Edition)     Full-text available via subscription   (Followers: 1)
Actualites Pharmaceutiques     Full-text available via subscription   (Followers: 7, SJR: 0.19, CiteScore: 0)
Actualites Pharmaceutiques Hospitalieres     Full-text available via subscription   (Followers: 3)
Acupuncture and Related Therapies     Hybrid Journal   (Followers: 8)
Acute Pain     Full-text available via subscription   (Followers: 15, SJR: 2.671, CiteScore: 5)
Ad Hoc Networks     Hybrid Journal   (Followers: 11, SJR: 0.53, CiteScore: 4)
Addictive Behaviors     Hybrid Journal   (Followers: 17, SJR: 1.29, CiteScore: 3)
Addictive Behaviors Reports     Open Access   (Followers: 9, SJR: 0.755, CiteScore: 2)
Additive Manufacturing     Hybrid Journal   (Followers: 11, SJR: 2.611, CiteScore: 8)
Additives for Polymers     Full-text available via subscription   (Followers: 23)
Advanced Drug Delivery Reviews     Hybrid Journal   (Followers: 175, SJR: 4.09, CiteScore: 13)
Advanced Engineering Informatics     Hybrid Journal   (Followers: 12, SJR: 1.167, CiteScore: 4)
Advanced Powder Technology     Hybrid Journal   (Followers: 17, SJR: 0.694, CiteScore: 3)
Advances in Accounting     Hybrid Journal   (Followers: 9, SJR: 0.277, CiteScore: 1)
Advances in Agronomy     Full-text available via subscription   (Followers: 16, SJR: 2.384, CiteScore: 5)
Advances in Anesthesia     Full-text available via subscription   (Followers: 28, SJR: 0.126, CiteScore: 0)
Advances in Antiviral Drug Design     Full-text available via subscription   (Followers: 2)
Advances in Applied Mathematics     Full-text available via subscription   (Followers: 10, SJR: 0.992, CiteScore: 1)
Advances in Applied Mechanics     Full-text available via subscription   (Followers: 11, SJR: 1.551, CiteScore: 4)
Advances in Applied Microbiology     Full-text available via subscription   (Followers: 24, SJR: 2.089, CiteScore: 5)
Advances In Atomic, Molecular, and Optical Physics     Full-text available via subscription   (Followers: 14, SJR: 0.572, CiteScore: 2)
Advances in Biological Regulation     Hybrid Journal   (Followers: 4, SJR: 2.61, CiteScore: 7)
Advances in Botanical Research     Full-text available via subscription   (Followers: 2, SJR: 0.686, CiteScore: 2)
Advances in Cancer Research     Full-text available via subscription   (Followers: 32, SJR: 3.043, CiteScore: 6)
Advances in Carbohydrate Chemistry and Biochemistry     Full-text available via subscription   (Followers: 9, SJR: 1.453, CiteScore: 2)
Advances in Catalysis     Full-text available via subscription   (Followers: 5, SJR: 1.992, CiteScore: 5)
Advances in Cell Aging and Gerontology     Full-text available via subscription   (Followers: 4)
Advances in Cellular and Molecular Biology of Membranes and Organelles     Full-text available via subscription   (Followers: 14)
Advances in Chemical Engineering     Full-text available via subscription   (Followers: 28, SJR: 0.156, CiteScore: 1)
Advances in Child Development and Behavior     Full-text available via subscription   (Followers: 10, SJR: 0.713, CiteScore: 1)
Advances in Chronic Kidney Disease     Full-text available via subscription   (Followers: 10, SJR: 1.316, CiteScore: 2)
Advances in Clinical Chemistry     Full-text available via subscription   (Followers: 26, SJR: 1.562, CiteScore: 3)
Advances in Colloid and Interface Science     Full-text available via subscription   (Followers: 20, SJR: 1.977, CiteScore: 8)
Advances in Computers     Full-text available via subscription   (Followers: 14, SJR: 0.205, CiteScore: 1)
Advances in Dermatology     Full-text available via subscription   (Followers: 15)
Advances in Developmental Biology     Full-text available via subscription   (Followers: 13)
Advances in Digestive Medicine     Open Access   (Followers: 11)
Advances in DNA Sequence-Specific Agents     Full-text available via subscription   (Followers: 7)
Advances in Drug Research     Full-text available via subscription   (Followers: 26)
Advances in Ecological Research     Full-text available via subscription   (Followers: 43, SJR: 2.524, CiteScore: 4)
Advances in Engineering Software     Hybrid Journal   (Followers: 29, SJR: 1.159, CiteScore: 4)
Advances in Experimental Biology     Full-text available via subscription   (Followers: 8)
Advances in Experimental Social Psychology     Full-text available via subscription   (Followers: 49, SJR: 5.39, CiteScore: 8)
Advances in Exploration Geophysics     Full-text available via subscription   (Followers: 1)
Advances in Fluorine Science     Full-text available via subscription   (Followers: 9)
Advances in Food and Nutrition Research     Full-text available via subscription   (Followers: 61, SJR: 0.591, CiteScore: 2)
Advances in Fuel Cells     Full-text available via subscription   (Followers: 17)
Advances in Genetics     Full-text available via subscription   (Followers: 20, SJR: 1.354, CiteScore: 4)
Advances in Genome Biology     Full-text available via subscription   (Followers: 10, SJR: 12.74, CiteScore: 13)
Advances in Geophysics     Full-text available via subscription   (Followers: 6, SJR: 1.193, CiteScore: 3)
Advances in Heat Transfer     Full-text available via subscription   (Followers: 24, SJR: 0.368, CiteScore: 1)
Advances in Heterocyclic Chemistry     Full-text available via subscription   (Followers: 12, SJR: 0.749, CiteScore: 3)
Advances in Human Factors/Ergonomics     Full-text available via subscription   (Followers: 23)
Advances in Imaging and Electron Physics     Full-text available via subscription   (Followers: 2, SJR: 0.193, CiteScore: 0)
Advances in Immunology     Full-text available via subscription   (Followers: 36, SJR: 4.433, CiteScore: 6)
Advances in Inorganic Chemistry     Full-text available via subscription   (Followers: 10, SJR: 1.163, CiteScore: 2)
Advances in Insect Physiology     Full-text available via subscription   (Followers: 2, SJR: 1.938, CiteScore: 3)
Advances in Integrative Medicine     Hybrid Journal   (Followers: 6, SJR: 0.176, CiteScore: 0)
Advances in Intl. Accounting     Full-text available via subscription   (Followers: 3)
Advances in Life Course Research     Hybrid Journal   (Followers: 8, SJR: 0.682, CiteScore: 2)
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: 18, SJR: 0.88, CiteScore: 2)
Advances in Mathematics     Full-text available via subscription   (Followers: 11, SJR: 3.027, CiteScore: 2)
Advances in Medical Sciences     Hybrid Journal   (Followers: 7, SJR: 0.694, CiteScore: 2)
Advances in Medicinal Chemistry     Full-text available via subscription   (Followers: 5)
Advances in Microbial Physiology     Full-text available via subscription   (Followers: 4, SJR: 1.158, CiteScore: 3)
Advances in Molecular and Cell Biology     Full-text available via subscription   (Followers: 23)
Advances in Molecular and Cellular Endocrinology     Full-text available via subscription   (Followers: 8)
Advances in Molecular Toxicology     Full-text available via subscription   (Followers: 7, SJR: 0.182, CiteScore: 0)
Advances in Nanoporous Materials     Full-text available via subscription   (Followers: 4)
Advances in Oncobiology     Full-text available via subscription   (Followers: 2)
Advances in Organ Biology     Full-text available via subscription   (Followers: 2)
Advances in Organometallic Chemistry     Full-text available via subscription   (Followers: 17, SJR: 1.875, CiteScore: 4)
Advances in Parallel Computing     Full-text available via subscription   (Followers: 7, SJR: 0.174, CiteScore: 0)
Advances in Parasitology     Full-text available via subscription   (Followers: 5, SJR: 1.579, CiteScore: 4)
Advances in Pediatrics     Full-text available via subscription   (Followers: 25, SJR: 0.461, CiteScore: 1)
Advances in Pharmaceutical Sciences     Full-text available via subscription   (Followers: 12)
Advances in Pharmacology     Full-text available via subscription   (Followers: 16, SJR: 1.536, CiteScore: 3)
Advances in Physical Organic Chemistry     Full-text available via subscription   (Followers: 8, SJR: 0.574, CiteScore: 1)
Advances in Phytomedicine     Full-text available via subscription  
Advances in Planar Lipid Bilayers and Liposomes     Full-text available via subscription   (Followers: 3, SJR: 0.109, CiteScore: 1)
Advances in Plant Biochemistry and Molecular Biology     Full-text available via subscription   (Followers: 10)
Advances in Plant Pathology     Full-text available via subscription   (Followers: 5)
Advances in Porous Media     Full-text available via subscription   (Followers: 5)
Advances in Protein Chemistry     Full-text available via subscription   (Followers: 19)
Advances in Protein Chemistry and Structural Biology     Full-text available via subscription   (Followers: 20, SJR: 0.791, CiteScore: 2)
Advances in Psychology     Full-text available via subscription   (Followers: 66)
Advances in Quantum Chemistry     Full-text available via subscription   (Followers: 6, SJR: 0.371, CiteScore: 1)
Advances in Radiation Oncology     Open Access   (Followers: 1, SJR: 0.263, CiteScore: 1)
Advances in Small Animal Medicine and Surgery     Hybrid Journal   (Followers: 3, SJR: 0.101, CiteScore: 0)
Advances in Space Biology and Medicine     Full-text available via subscription   (Followers: 6)
Advances in Space Research     Full-text available via subscription   (Followers: 414, SJR: 0.569, CiteScore: 2)
Advances in Structural Biology     Full-text available via subscription   (Followers: 5)
Advances in Surgery     Full-text available via subscription   (Followers: 12, SJR: 0.555, CiteScore: 2)
Advances in the Study of Behavior     Full-text available via subscription   (Followers: 35, SJR: 2.208, CiteScore: 4)
Advances in Veterinary Medicine     Full-text available via subscription   (Followers: 19)
Advances in Veterinary Science and Comparative Medicine     Full-text available via subscription   (Followers: 15)
Advances in Virus Research     Full-text available via subscription   (Followers: 5, SJR: 2.262, CiteScore: 5)
Advances in Water Resources     Hybrid Journal   (Followers: 49, SJR: 1.551, CiteScore: 3)
Aeolian Research     Hybrid Journal   (Followers: 6, SJR: 1.117, CiteScore: 3)
Aerospace Science and Technology     Hybrid Journal   (Followers: 363, SJR: 0.796, CiteScore: 3)
AEU - Intl. J. of Electronics and Communications     Hybrid Journal   (Followers: 8, SJR: 0.42, CiteScore: 2)
African J. of Emergency Medicine     Open Access   (Followers: 6, SJR: 0.296, CiteScore: 0)
Ageing Research Reviews     Hybrid Journal   (Followers: 11, SJR: 3.671, CiteScore: 9)
Aggression and Violent Behavior     Hybrid Journal   (Followers: 471, SJR: 1.238, CiteScore: 3)
Agri Gene     Hybrid Journal   (Followers: 1, SJR: 0.13, CiteScore: 0)
Agricultural and Forest Meteorology     Hybrid Journal   (Followers: 17, SJR: 1.818, CiteScore: 5)
Agricultural Systems     Hybrid Journal   (Followers: 31, SJR: 1.156, CiteScore: 4)
Agricultural Water Management     Hybrid Journal   (Followers: 43, SJR: 1.272, CiteScore: 3)
Agriculture and Agricultural Science Procedia     Open Access   (Followers: 4)
Agriculture and Natural Resources     Open Access   (Followers: 3)
Agriculture, Ecosystems & Environment     Hybrid Journal   (Followers: 58, SJR: 1.747, CiteScore: 4)
Ain Shams Engineering J.     Open Access   (Followers: 5, SJR: 0.589, CiteScore: 3)
Air Medical J.     Hybrid Journal   (Followers: 6, SJR: 0.26, CiteScore: 0)
AKCE Intl. J. of Graphs and Combinatorics     Open Access   (SJR: 0.19, CiteScore: 0)
Alcohol     Hybrid Journal   (Followers: 12, SJR: 1.153, CiteScore: 3)
Alcoholism and Drug Addiction     Open Access   (Followers: 11)
Alergologia Polska : Polish J. of Allergology     Full-text available via subscription   (Followers: 1)
Alexandria Engineering J.     Open Access   (Followers: 2, SJR: 0.604, CiteScore: 3)
Alexandria J. of Medicine     Open Access   (Followers: 1, SJR: 0.191, CiteScore: 1)
Algal Research     Partially Free   (Followers: 11, SJR: 1.142, CiteScore: 4)
Alkaloids: Chemical and Biological Perspectives     Full-text available via subscription   (Followers: 2)
Allergologia et Immunopathologia     Full-text available via subscription   (Followers: 1, SJR: 0.504, CiteScore: 1)
Allergology Intl.     Open Access   (Followers: 5, SJR: 1.148, CiteScore: 2)
Alpha Omegan     Full-text available via subscription   (SJR: 3.521, CiteScore: 6)
ALTER - European J. of Disability Research / Revue Européenne de Recherche sur le Handicap     Full-text available via subscription   (Followers: 10, SJR: 0.201, CiteScore: 1)
Alzheimer's & Dementia     Hybrid Journal   (Followers: 52, SJR: 4.66, CiteScore: 10)
Alzheimer's & Dementia: Diagnosis, Assessment & Disease Monitoring     Open Access   (Followers: 4, SJR: 1.796, CiteScore: 4)
Alzheimer's & Dementia: Translational Research & Clinical Interventions     Open Access   (Followers: 4, SJR: 1.108, CiteScore: 3)
Ambulatory Pediatrics     Hybrid Journal   (Followers: 6)
American Heart J.     Hybrid Journal   (Followers: 57, SJR: 3.267, CiteScore: 4)
American J. of Cardiology     Hybrid Journal   (Followers: 62, SJR: 1.93, CiteScore: 3)
American J. of Emergency Medicine     Hybrid Journal   (Followers: 44, SJR: 0.604, CiteScore: 1)
American J. of Geriatric Pharmacotherapy     Full-text available via subscription   (Followers: 11)
American J. of Geriatric Psychiatry     Hybrid Journal   (Followers: 13, SJR: 1.524, CiteScore: 3)
American J. of Human Genetics     Hybrid Journal   (Followers: 34, SJR: 7.45, CiteScore: 8)
American J. of Infection Control     Hybrid Journal   (Followers: 29, SJR: 1.062, CiteScore: 2)
American J. of Kidney Diseases     Hybrid Journal   (Followers: 35, SJR: 2.973, CiteScore: 4)
American J. of Medicine     Hybrid Journal   (Followers: 48)
American J. of Medicine Supplements     Full-text available via subscription   (Followers: 3, SJR: 1.967, CiteScore: 2)
American J. of Obstetrics and Gynecology     Hybrid Journal   (Followers: 232, SJR: 2.7, CiteScore: 4)
American J. of Ophthalmology     Hybrid Journal   (Followers: 66, SJR: 3.184, CiteScore: 4)
American J. of Ophthalmology Case Reports     Open Access   (Followers: 5, SJR: 0.265, CiteScore: 0)
American J. of Orthodontics and Dentofacial Orthopedics     Full-text available via subscription   (Followers: 6, SJR: 1.289, CiteScore: 1)
American J. of Otolaryngology     Hybrid Journal   (Followers: 25, SJR: 0.59, CiteScore: 1)
American J. of Pathology     Hybrid Journal   (Followers: 29, SJR: 2.139, CiteScore: 4)
American J. of Preventive Medicine     Hybrid Journal   (Followers: 28, SJR: 2.164, CiteScore: 4)
American J. of Surgery     Hybrid Journal   (Followers: 39, SJR: 1.141, CiteScore: 2)
American J. of the Medical Sciences     Hybrid Journal   (Followers: 12, SJR: 0.767, CiteScore: 1)
Ampersand : An Intl. J. of General and Applied Linguistics     Open Access   (Followers: 7)
Anaerobe     Hybrid Journal   (Followers: 4, SJR: 1.144, CiteScore: 3)
Anaesthesia & Intensive Care Medicine     Full-text available via subscription   (Followers: 63, SJR: 0.138, CiteScore: 0)
Anaesthesia Critical Care & Pain Medicine     Full-text available via subscription   (Followers: 20, SJR: 0.411, CiteScore: 1)
Anales de Cirugia Vascular     Full-text available via subscription   (Followers: 1)
Anales de Pediatría     Full-text available via subscription   (Followers: 3, SJR: 0.277, CiteScore: 0)
Anales de Pediatría (English Edition)     Full-text available via subscription  
Anales de Pediatría Continuada     Full-text available via subscription  
Analytic Methods in Accident Research     Hybrid Journal   (Followers: 5, SJR: 4.849, CiteScore: 10)
Analytica Chimica Acta     Hybrid Journal   (Followers: 44, SJR: 1.512, CiteScore: 5)
Analytica Chimica Acta : X     Open Access  
Analytical Biochemistry     Hybrid Journal   (Followers: 200, SJR: 0.633, CiteScore: 2)
Analytical Chemistry Research     Open Access   (Followers: 12, SJR: 0.411, CiteScore: 2)
Analytical Spectroscopy Library     Full-text available via subscription   (Followers: 14)
Anesthésie & Réanimation     Full-text available via subscription   (Followers: 2)
Anesthesiology Clinics     Full-text available via subscription   (Followers: 23, SJR: 0.683, CiteScore: 2)
Angiología     Full-text available via subscription   (SJR: 0.121, CiteScore: 0)
Angiologia e Cirurgia Vascular     Open Access   (Followers: 1, SJR: 0.111, CiteScore: 0)
Animal Behaviour     Hybrid Journal   (Followers: 207, SJR: 1.58, CiteScore: 3)
Animal Feed Science and Technology     Hybrid Journal   (Followers: 6, SJR: 0.937, CiteScore: 2)

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Similar Journals
Journal Cover
Acta Haematologica Polonica
Journal Prestige (SJR): 0.128
Number of Followers: 1  

  Free journal Free journal
ISSN (Print) 0001-5814
Published by Elsevier Homepage  [3160 journals]
  • Artykuł redakcyjny/Editorial
    • Abstract: Publication date: October–December 2017
      Source:Acta Haematologica Polonica, Volume 48, Issue 4

      PubDate: 2017-12-27T00:02:04Z
  • Analiza skuteczności ibrutynibu w podgrupie chorych na przewlekłą
           białaczkę limfocytową z delecją 17p: badanie obserwacyjne Polskiej
           Grupy ds. Leczenia Białaczek u Dorosłych (PALG)
    • Authors: Bartosz Puła; Elżbieta Iskierka-Jażdżewska; Marek Hus; Agnieszka Szymczyk; Aleksandra Gołos; Magdalena Piotrowska; Daria Zawirska; Jan Maciej Zaucha; Paweł Steckiewicz; Marcin Pasiarski; Dominik Chraniuk; Weronika Piszczek; Michał Osowiecki; Edyta Subocz; Janusz Hałka; Anna Waszczuk-Gajda; Joanna Drozd-Sokołowska; Wanda Knopińka-Posłuszny; Marek Dudziński; Jadwiga Hołojda; Małgorzata Wojciechowska; Waldemar Kulikowski; Agnieszka Szeremet; Beata Kumięga; Andrzej Pluta; Mirosław Markiewicz; Krzysztof Giannopoulos; Tadeusz Robak; Krzysztof Warzocha; Krzysztof Jamroziak
      Abstract: Publication date: Available online 10 November 2017
      Source:Acta Haematologica Polonica
      Author(s): Bartosz Puła, Elżbieta Iskierka-Jażdżewska, Marek Hus, Agnieszka Szymczyk, Aleksandra Gołos, Magdalena Piotrowska, Daria Zawirska, Jan Maciej Zaucha, Paweł Steckiewicz, Marcin Pasiarski, Dominik Chraniuk, Weronika Piszczek, Michał Osowiecki, Edyta Subocz, Janusz Hałka, Anna Waszczuk-Gajda, Joanna Drozd-Sokołowska, Wanda Knopińka-Posłuszny, Marek Dudziński, Jadwiga Hołojda, Małgorzata Wojciechowska, Waldemar Kulikowski, Agnieszka Szeremet, Beata Kumięga, Andrzej Pluta, Mirosław Markiewicz, Krzysztof Giannopoulos, Tadeusz Robak, Krzysztof Warzocha, Krzysztof Jamroziak
      Background The 17p deletion is regarded as the strongest poor prognostic factor in chronic lymphocytic leukemia (CLL). Results of recently performed clinical trials have suggested that ibrutinib significantly improves the outcome in this patient group. Aim The study aimed at analyzing the efficacy and adverse events profile of ibrutinib monotherapy in CLL patients with 17p deletion treated in routine clinical practice outside clinical trials. Materials and Methods Clinical response and adverse events profile of ibrutinib monotherapy were assessed in thirty five CLL patients with 17p treated within the ibrutinib named patients program in Poland. Results Overall response rate was 80% (28/35 patients) with median observation time of 24.2 months (range 0,1 – 30,9). Complete remission was observed in 5 patients (14.3%), partial remission in 11 (31.4%), partial remission with lymphocytosis in 13 (37.1%), whereas stable disease and progression was noted in 4 (11.4%) and 1 (2.9%) respectively. Response was not assessed in 1 patient. Median progression-free survival was 29.5 months, whereas median overall survival was not reached. Eleven patients died (7 because of infection, 1 of CLL progression, 1 of sudden cardiac death, 1 of disseminated breast cancer and 1 of unknown causes). In 13 patients (37.1%) at least one 3 or 4 grade adverse event occurred. In 11 patients (31.4%) the treatment was temporary withheld or the dose reduced due to adverse events. Conclusion Ibrutinib is characterized by high clinical efficacy and acceptable toxicity in CLL patients with 17p deletion in daily clinical practice.

      PubDate: 2017-11-16T09:48:08Z
      DOI: 10.1016/j.achaem.2017.10.004
  • Choroba Gauchera – zalecenia dotyczące rozpoznawania, leczenia
           i monitorowania
    • Authors: Piotr Hasiński; Mirosław Bik-Multanowski; Magdalena Koba-Wszędobył; Mieczysław Walczak; Marek Bubnowski; Agnieszka Milewska-Kranc; Andrzej Smyk; Maciej Machaczka
      Abstract: Publication date: Available online 5 November 2017
      Source:Acta Haematologica Polonica
      Author(s): Piotr Hasiński, Mirosław Bik-Multanowski, Magdalena Koba-Wszędobył, Mieczysław Walczak, Marek Bubnowski, Agnieszka Milewska-Kranc, Andrzej Smyk, Maciej Machaczka
      The following recommendations are the first complete document concerning the diagnosis, treatment and monitoring of Gaucher disease (GD) in Poland. GD is a rare, genetically determined storage disorder, involving deficiency or absence of glucocerebrosidase activity, a lysosomal enzyme that digests glucosylceramide. Glucosylceramide excessively accumulates in the monocyte-macrophage system (Gaucher cells), which in turn accumulate primarily in the bone marrow, spleen and liver. In the most severe forms of the disease, central nervous system is also involved. There are three clinical types of Gaucher disease, and the primary criteria for their differentiation is the involvement of the central nervous system, and the rate of symptom progression. The least severe and most common is type 1 GD, characterised mainly by hematological manifestations, such as thrombocytopenia and anemia, splenomegaly, hepatomegaly and skeletal involvement (bone pain, bone deformities, pathological fractures). Types 2 and 3 GD involve various central nervous system manifestations. Diagnosis of Gaucher disease is difficult, particularly in individuals from families without prior GD history. Gaucher disease is characterised by a heterogenic disease course and is rarely considered in differential diagnosis. This presents a major problem, as correct diagnosis and treatment implementation are often delayed by many years. This can lead to serious complications and even premature death of the patient. Currently, the primary therapeutic approach is an enzyme replacement therapy, involving an intravenous administration of recombinant glucocerebrosidase. Treatment of mild and moderate forms of the disease also includes an oral therapy which inhibits the production of glucocerebroside.

      PubDate: 2017-11-08T10:53:30Z
      DOI: 10.1016/j.achaem.2017.10.001
  • Strategie optymalizacji terapii lenalidomidem u chorych na szpiczaka
    • Authors: Agnieszka Szymczyk; Iwona Hus
      Abstract: Publication date: Available online 4 November 2017
      Source:Acta Haematologica Polonica
      Author(s): Agnieszka Szymczyk, Iwona Hus
      The introduction of new drugs (immunomodulators, proteasome inhibitors) into the treatment of multiple myeloma (MM) has contributed to the increase in response rates and also to a significant prolongation of overall survival, allowing multiple myeloma to become a chronic disease. Lenalidomide is an immunomodulatory agent, more effective and less toxic than the first immunomodulatory drug introduced into MM therapy - thalidomide. Lenalidomide should be given continously and longer treatment is associated with increased response rates and improved quality of response. That is why prophylaxis and proper management of adverse events is extremely important for continuation of the therapy. The dose of lenalidomide should be modified in patients with renal failure and cytopenias, also effective venous thromboembolism (VTE) is required. This article describes optimization of lenalidomide therapy with regard to the most common clinical problems.

      PubDate: 2017-11-08T10:53:30Z
      DOI: 10.1016/j.achaem.2017.10.002
  • Propozycje postępowania z chorymi na nowotwory krwi, którzy przechodzą
           spod opieki hematologów pediatrycznych pod opiekę hematologów
    • Authors: Jan Styczyński; Iwona Hus; Katarzyna Derwich; Tomasz Szczepański; Lidia Gil; Tadeusz Robak; Elżbieta Kasprowicz; Wiesław W. Jędrzejczak
      Abstract: Publication date: Available online 20 October 2017
      Source:Acta Haematologica Polonica
      Author(s): Jan Styczyński, Iwona Hus, Katarzyna Derwich, Tomasz Szczepański, Lidia Gil, Tadeusz Robak, Elżbieta Kasprowicz, Wiesław W. Jędrzejczak
      Background Patients cured from hematological malignancies should be subject to long-life routine medical supervision. Patients who did not complete their therapy during childhood require further therapy when reaching adulthood (18 years). Objective Determination of guidelines and suggestions on proceeding with pediatric patients with hematological malignancies undergoing transition to adult hematology centers, with respect to Polish conditions of national medical health-care system. Methods Panel of experts in the field of pediatric and adult hematology analyzed current situation in Polish pediatric and adult hematology centers and reviewed the literature on transition process of minor patients from pediatric to adult setting. Results Factors determining problems of patient transition and models of care of patient in pediatric and adult hematology settings were analyzed. Existing models of transition in Poland as well as experience from international models were presented: model of direct transition, model of adolescence medicine, sequential model and consultation model. Suggestions of changes facilitating process of patient transition, and guidelines on proceeding with patients with respect to diagnosis of malignancy and phase of oncological therapy were presented, involving cooperation of pediatric and adult centers, specialists in pediatric hematology and adult hematology, as well as the payer and scientific societies. Conclusions Process of transition of patients requires coordination and cooperation, preferably at the level of medical institutions. This should ensure optimal medical care to patients cured from malignancy and facilitate long-term medical supervision and gathering data on treatment efficacy and side effects.

      PubDate: 2017-10-24T22:20:42Z
      DOI: 10.1016/j.achaem.2017.09.001
  • Nieinterwencyjne badanie oceniające odpowiedź na leczenie u pacjentów z
           przewlekłą białaczką limfocytową (CLL) w zależności od
           zastosowanego schematu rytuksymab-chemioterapia
    • Authors: Krzysztof Warzocha; Marta Fidecka; Grzegorz Mazur; Daria Zawirska; Magdalena Piotrowska
      Abstract: Publication date: Available online 19 October 2017
      Source:Acta Haematologica Polonica
      Author(s): Krzysztof Warzocha, Marta Fidecka, Grzegorz Mazur, Daria Zawirska, Magdalena Piotrowska
      A multicenter, prospective, observational, non-interventional study evaluated the response to treatment in patients with chronic lymphocytic leukemia (CLL) depending on the rituximab-chemotherapy (R-chemo) regimen used in routine clinical practice in Poland. The primary endpoint was the overall response rate (ORR) regarding the R-chemo regimen. The secondary endpoints were complete remission (CR), partial remission (PR), progressive disease (PD) and stable disease (SD) rates. A total of 400 CLL patients were enrolled in 20 sites in Poland. Data were collected on-line using eCRF during 6-months rituximab treatment. There were 150 treatment-naive patients (37.5%) and 250 patients with recurrent disease (62.5%) enrolled to the study. The overall ORR in R-FC-treated patients was 87.28%, 78.57% for the R-COP regimen, and for other regimens – 51.72%. Complete remission was achieved in 141 patients (36.25%), including 16.40% in patients receiving first-line treatment and 11.76% in relapsed disease. Partial remission was achieved in 166 patients (42.67%), including 38.4% in patients on first-line treatment and 38.56% in patients treated for recurrence. Complete or partial responses were achieved in 78.92% in total. No new safety signals were detected in comparison to safety profile described in Summary of Product Characteristics.

      PubDate: 2017-10-24T22:20:42Z
      DOI: 10.1016/j.achaem.2017.09.002
  • Indolent systemic mastocytosis associated with multiple myeloma: A rare
    • Authors: Karolina Chromik; Grzegorz Helbig Joanna Dziaczkowska-Suszek Anna Krzysztof
      Abstract: Publication date: Available online 9 October 2017
      Source:Acta Haematologica Polonica
      Author(s): Karolina Chromik, Grzegorz Helbig, Joanna Dziaczkowska-Suszek, Anna Kopińska, Krzysztof Woźniczka, Sławomira Kyrcz-Krzemień
      Systemic mastocytosis (SM) includes a wide spectrum of clonal disorders characterized by an abnormal growth and accumulation of mast cells. SM may be associated with other hematological neoplasms (SM-AHN) among them the myeloproliferative neoplasms and myelodysplastic syndromes are the most common. The coexistence of SM with lymphoid malignancies has rarely been reported so far. The occurrence of SM associated with multiple myeloma (MM) is extremely rare and its prognosis remains unclear. The treatment of SM-AHM requires an individual approach. We report a male patient diagnosed with indolent SM associated with MM. He did not require the therapy for his SM, but started the treatment against MM. He received the induction regiment consisting of bortezomibe, thalidomide and dexamethasone (VTD). After six cycles of VTD he achieved a very good partial response, but refused autologous stem cell transplantation as response consolidation and eventually died of myeloma progression a couple months later. Herein we discuss the likely pathophysiologic mechanisms underlying those two separate entities.

      PubDate: 2017-10-11T17:25:34Z
  • Stany chorobowe związane z nadmiernym gromadzeniem żelaza oraz metody
           oznaczania zawartości żelaza w wątrobie
    • Authors: Cyprian Olchowy; Jowita Marcin Daria Anna Olchowy Adam Urszula Zaleska-Dorobisz
      Abstract: Publication date: Available online 6 October 2017
      Source:Acta Haematologica Polonica
      Author(s): Cyprian Olchowy, Jowita Frączkiewicz, Marcin Pawłowski, Daria Smoląg, Anna Olchowy, Adam Maślak, Urszula Zaleska-Dorobisz, Krzysztof Kałwak
      Iron overload refers to the excessive accumulation of iron in different organs. It occurs in the course of increased intestinal absorption in hereditary hemochromatosis, repeated blood transfusions, or liver diseases. Excess of iron exerts toxic effect on internal organs leading to liver damage resulting in liver cirrhosis, liver failure, or hepatocellular carcinoma among others. Assessment of liver iron content is essential for diagnosing diseases caused by iron overload and monitoring of iron-reducing therapy. A biopsy is a gold standard of assessment of liver iron concentration; however, new techniques based on the use of magnetic resonance imaging (MRI) gain in importance, as they are non-invasive, reliable, and enable to evaluate iron content in areas inaccessible by biopsy such as brain. This article reviews the currently used methods for assessment of liver iron overload, especially using MRI, in terms of their clinical usefulness and accuracy in different diseases. The authors conclude that assessment of iron overload is difficult, especially in patients with very high or very low level of iron and should be conducted taking into account the patient's clinical condition and the course of primary disease. Increase in the popularity of magnetic resonance signal intensity ratio (SIR) results from its safety, relatively low costs, availability, and possibility to obtain reliable results.

      PubDate: 2017-10-11T17:25:34Z
  • Vitamin C as a modulator of oxidative stress in erythrocytes of stored
    • Authors: Soumya Ravikumar; Vani Rajashekharaiah
      Abstract: Publication date: Available online 6 October 2017
      Source:Acta Haematologica Polonica
      Author(s): Soumya Ravikumar, Vani Rajashekharaiah
      Aim To determine the effects of Vitamin C (VC-ascorbic acid) as an additive on erythrocytes of stored blood. Background Oxidative stress (OS) plays a major role in the formation of storage lesion of erythrocytes. Antioxidants, such as VC could be beneficial in combating oxidative damage during storage. Materials and methods Blood obtained from male Wistar rats was stored at 4°C in anticoagulant solution citrate-phosphate-dextrose-adenine solution. Blood samples were divided into 3 groups – (i) Controls, (ii) VC 10 (VC at a concentration of 10mM), (iii) VC 30 (VC at a concentration of 30mM). Markers of OS in erythrocytes such as – hemoglobin, superoxides, antioxidant enzymes (superoxide dismutase; SOD, catalase and glutathione peroxidase), hemolysis, lipid peroxidation products (conjugate dienes and malondialdehyde), protein oxidation products and ascorbic acid were determined on days 0, 10 and 15 of storage. Results Addition of ascorbic acid to the storage solution contributed to the protection of erythrocytes from oxidative damage. Ascorbic acid at a concentration of 30mM decreased SOD levels and increased protein sulfhydryls (P-SH) levels on day 15 showing that higher concentration supplemented the inherent antioxidant defense system of erythrocytes during blood storage. Conclusion VC proved to be effective in combating OS during blood storage. However, further exploration of antioxidants as additives and the erythrocyte storage lesion would result in better management of blood storage.

      PubDate: 2017-10-11T17:25:34Z
  • Zaburzenia genetyczne u dzieci z ostrą białaczką limfoblastyczną, ich
           znaczenie kliniczne i terapeutyczne
    • Authors: Magdalena Romiszewska; Iwona Malinowska
      Abstract: Publication date: Available online 3 September 2017
      Source:Acta Haematologica Polonica
      Author(s): Magdalena Romiszewska, Iwona Malinowska
      Acute lymphoblastic leukaemia (ALL) is the most common paediatric malignancy. Despite the fact that the outcome of treatment of ALL in children has improved in the last decades, still as many as 20% of children will undergo treatment failure. Over the last years, there have been great efforts to characterize the genetic alterations of leukaemic cells, which could affect the clinical course of the disease and become new markers that may be targeted with novel therapies. Genomic profiling and sequencing studies have not only identified new subtypes of ALL (e.g. Philadelphia chromosome-like ALL, early T-cell precursor ALL) but also helped to understand the genetic basis of leukaemogenesis and predict treatment failure. Those efforts contributed not only to better risk stratification of children with ALL, but also to development of new tailored therapeutic strategies. So far, improvements in survival of children with Ph+ ALL (Philadelphia positive ALL) have been demonstrated due to combination of intensive chemotherapy with tyrosine kinase (TK) inhibitor (imatinib). There are also some pre- and clinical testing of other inhibitors (TK, FLT3, PI3K/mTOR, MEK) and specific antibodies (CD19 and CD22). Widespread use of modern diagnostic techniques at the early stage of the disease would enable rapid identification of children with a high-risk leukaemia and use of more intensive and tailored therapy.

      PubDate: 2017-09-08T05:54:07Z
      DOI: 10.1016/j.achaem.2017.06.003
  • Standardy diagnostyki oraz nowe trendy w leczeniu ostrej białaczki
    • Authors: Dagmara Szmajda; Ewa Balcerczak; Adrian Krygier
      Abstract: Publication date: Available online 1 September 2017
      Source:Acta Haematologica Polonica
      Author(s): Dagmara Szmajda, Ewa Balcerczak, Adrian Krygier
      Acute myeloid leukemia (AML) is the most common cancer of white blood cells in adults. Men over 65 years old are more prone to develop this disease. Symptoms that lead patients to visit the doctor are: high fever, bone pain, weakness and signs of infection. The etiology of AML is not yet fully understood. The predisposing factors for acute myeloid leukemia may include environmental and genetic factors. If left untreated, it can lead to death within a few weeks. Therefore, it is important to quickly identify the disease and to implement appropriate treatment, which will allow to increase the percentage of survival among patients. The basis of AML diagnosis is the presence of more than 20% of blasts in blood or bone marrow smears. The choice of AML treatment depends on prognostic factors: patients’ age and sex and cytogenetic-molecular risk. The treatment regimen for AML is outdated and remains almost unchanged for over 30 years. Understanding the molecular basis of this disease development and pathomechanism allows to search for new effective treatments, often based on targeted therapies. This article presents contemporary standards of AML diagnosis and the latest trends in its treatment.

      PubDate: 2017-09-02T03:58:38Z
      DOI: 10.1016/j.achaem.2017.07.009
  • Genomic landscape of human erythroleukemia K562 cell line, as determined
           by next-generation sequencing and cytogenetics
    • Authors: Małgorzata Kurkowiak; Monika Pępek; Marcin M. Machnicki; Iwona Solarska; Katarzyna Borg; Małgorzata Rydzanicz; Piotr Stawiński; Rafał Płoski; Tomasz Stokłosa
      Abstract: Publication date: Available online 24 August 2017
      Source:Acta Haematologica Polonica
      Author(s): Małgorzata Kurkowiak, Monika Pępek, Marcin M. Machnicki, Iwona Solarska, Katarzyna Borg, Małgorzata Rydzanicz, Piotr Stawiński, Rafał Płoski, Tomasz Stokłosa
      We have performed detailed analysis of the genomic landscape of commercially available K562 cells, employing targeted enrichment of nearly 1300 cancer-related genes followed by next-generation sequencing (NGS) and also classical cytogenetics. Deep sequencing revealed 88 variants of potentially biological significance. Among them we have detected alterations in genes already known to be mutated in K562, such as TP53 but also in several other genes, which are implicated in tumorigenesis and drug resistance, such as MLH1, ASXL1 and BRCA1 as the most prominent examples. Fluorescence in situ hybridization (FISH) of interphases of K562 cells revealed multiplication of the BCR and ABL1 gene copies, as well as the amplification of the BCR-ABL1 fusion gene. Our results may help to better understand genomic instability of the blastic phase of CML represented by the K562 cell line and can help researchers who want to employ this cell line in various experimental settings.

      PubDate: 2017-09-02T03:58:38Z
      DOI: 10.1016/j.achaem.2017.06.002
  • Extraneural relapse of medulloblastoma mimicking acute leukemia: A
           diagnostic challenge in adult patient
    • Authors: Monika Joks; Maciej Machaczka; Anna Czyż; Jolanta Parulska; Maciej Majcherek; Jan Bręborowicz; Grzegorz Dworacki; Mieczysław Komarnicki
      Abstract: Publication date: Available online 12 August 2017
      Source:Acta Haematologica Polonica
      Author(s): Monika Joks, Maciej Machaczka, Anna Czyż, Jolanta Parulska, Maciej Majcherek, Jan Bręborowicz, Grzegorz Dworacki, Mieczysław Komarnicki
      Medulloblastoma is the most frequent malignant tumor of the central nervous system (CNS) in children, but it can rarely occur in adults. Extraneural relapse of medulloblastoma occurs very rarely and it is usually associated with dismal prognosis. We present a case of young adult with relapsed medulloblastoma with extraneural metastases in the bone marrow and expression of terminal deoxynucleotidyl transferase (TdT) on the malignant cells mimicking acute leukemia. To the best of our knowledge, this is the first report of medulloblastoma exhibiting expression of the TdT in adult. We would like to emphasize that in cases like this, differential diagnosis of anemia and thrombocytopenia in adults should include a consideration of primary or secondary bone marrow involvement by medulloblastoma or other rare malignancy.

      PubDate: 2017-08-21T19:55:49Z
      DOI: 10.1016/j.achaem.2017.08.002
  • Rituximab-associated progressive multifocal leukoencephalopathy after a
           single cycle of R-CHOP for T-cell/histiocyte-rich large B-cell lymphoma
    • Authors: Moa Forssberg; Monika Klimkowska; Maciej Machaczka
      Abstract: Publication date: Available online 12 August 2017
      Source:Acta Haematologica Polonica
      Author(s): Moa Forssberg, Monika Klimkowska, Maciej Machaczka
      Progressive multifocal leukoencephalopathy (PML) is a disease of immunocompromised patients caused by reactivation of the John Cunningham polyomavirus (JCV). A monoclonal anti-CD20 antibody rituximab is widely used as an important part of therapy for B-cell non-Hodgkin lymphomas and various autoimmune diseases. It is not fully explained how rituximab reactivates JCV. In this report, we present the case of a 61-year-old man with T-cell/histiocyte-rich large B-cell lymphoma who was treated with R-CHOP and intrathecal methotrexate. Two weeks after the first R-CHOP course he developed dysarthria, diplopia, and disturbances in motor coordination. Based on CT/MRI results showing 3cm×2cm large hypodense white matter lesion in left cerebellar hemisphere, and detection of JCV in the cerebrospinal fluid (14300viral copies/mL), the patient was diagnosed with PML. Despite treatment attempt with cidofovir and IVIG, the patient's neurological status continued to worsen. He developed progressive motor neuron deficits but retained intact cognitive functions. The patient deceased nearly three months after onset of rituximab treatment. Rituximab is a milestone in treatment of many hematological and autoimmune diseases. Considering how widespread has the use of rituximab become, the overall risk of developing PML is relatively low. Nevertheless, since the end of 1990s several reports were published on PML development in association with usage of rituximab. The authors would like to emphasize that although the total risk of PML occurrence in patients treated with rituximab is low, it is important that physicians administrating rituximab therapy are aware of this serious complication.

      PubDate: 2017-08-21T19:55:49Z
      DOI: 10.1016/j.achaem.2017.08.003
  • Methods for detection of microparticles derived from blood and endothelial
    • Authors: Małgorzata Gradziuk; Piotr Radziwon
      Abstract: Publication date: Available online 5 August 2017
      Source:Acta Haematologica Polonica
      Author(s): Małgorzata Gradziuk, Piotr Radziwon

      PubDate: 2017-08-11T12:23:52Z
      DOI: 10.1016/j.achaem.2017.03.003
  • Aloplasty of an ankylosed knee in a patient with severe haemophilia A
    • Authors: Jerzy Mirosław Jaworski; Adam Zawojski; Joanna Zdziarska; Krzysztof Czernicki; Magdalena Wilk-Frańczuk
      Abstract: Publication date: Available online 29 July 2017
      Source:Acta Haematologica Polonica
      Author(s): Jerzy Mirosław Jaworski, Adam Zawojski, Joanna Zdziarska, Krzysztof Czernicki, Magdalena Wilk-Frańczuk
      The goal of this report is to describe a case of knee arthroplasty in a patient with over a 20 year long history of ankylosis of the left knee joint in the course of severe haemophilia A. Clinical and functional conditions were assessed by means of a Knee Society Score (KSS); pain intensity by Visual Analogue Scale (VAS); and intensity of degeneration of bone forming the joint by means of the Kellgren-Lawrence Scale. ROM of the knee joint before and after the operation was measured. We used hinge prosthesis of S-ROM by De Puy to restore the knee joint. As a result of the operation we got axial alignment of the limb. During two years of clinical follow-up, the patient achieved increasing mobility of the operated knee, full extension, and 80 degrees of active flexion. There were no radiological signs of loosening of the implant. The patients’ functional capabilities improved significantly; and pain occurred only occasionally.

      PubDate: 2017-08-01T10:30:42Z
      DOI: 10.1016/j.achaem.2017.07.008
  • Zastosowanie cytometrii przepływowej w badaniach
           immunohematologicznych krwinek czerwonych
    • Authors: Jadwiga Fabijańska-Mitek; Anna Stachurska
      Abstract: Publication date: Available online 27 July 2017
      Source:Acta Haematologica Polonica
      Author(s): Jadwiga Fabijańska-Mitek, Anna Stachurska
      Flow cytometry (FC) has been primarily applied to the diagnosis of hematological malignancies, and thereafter, to detection and quantification of CD34+ cells in bone marrow transplants, and granulocytes in neutropenias and paroxysmal nocturnal hemoglobinuria (PNH). In PNH and hereditary spherocytosis, changes in some of the erythrocyte membrane proteins are tested (CD59, CD55, and band 3). The purpose of this paper is to focus on the use of FC in RBC testing. With anti-D, -HbF, and -CA (carbonic anhydrase), we can detect RhD+, HbF+, and CA- fetal RBCs in the maternal RhD-, HbF-, and CA+ blood sample. Obtained results allow to select the appropriate dose of anti-D Ig in the RhD prophylaxis of feto-maternal incompatibility, or to detect the cause of fetal anemia. Expression of antigens and their weak variants, and concentration of specific antibodies, can also be assessed. It is possible to observe changes in selected CD molecules during storage of RBC units. If RBCs for transfusion are unavailable, due to patient's unusual antibody specificity, some of the available RBCs are opsonised, and then phagocytosis with the recipient monocytes is assessed. The microscopic time-consuming and subjective assay is usually used. Stained CD14+ monocytes and CD235a+ erythrocytes are visible on cytograms as well as their interaction. It makes evaluation of phagocytosis easier and objective. Microparticles of RBCs released during storage are also detected. They are 235a+. In differentiation of hemolysis causes, it is important to measure osmotic fragility, and that can be also achieved using FC. Flow cytometry should be applied to immunohematological testing of red blood cells more often than now.

      PubDate: 2017-08-01T10:30:42Z
      DOI: 10.1016/j.achaem.2017.07.005
  • Nietypowe objawy kliniczne szpiczaka plazmocytowego
    • Authors: Artur Jurczyszyn; Magdalena Olszewska-Szopa
      Abstract: Publication date: Available online 27 July 2017
      Source:Acta Haematologica Polonica
      Author(s): Artur Jurczyszyn, Magdalena Olszewska-Szopa
      Multiple myeloma accounting for 10% of malignancies of haematopoietic system is a heterogenous disease. In the era of individual approach to the patient, we are more and more interested in distinct features and forms of this malignancy. In the article, we described rare MM manifestations with reference to location (central nervous system and skin), M-protein produced by the tumor cells (biclonal and immunoglobulin M), early onset of the disease (young versus old) and pregnancy in MM patients.

      PubDate: 2017-08-01T10:30:42Z
      DOI: 10.1016/j.achaem.2017.07.004
  • Elevated serum concentrations of β-2-microglobulin are often found at the
           time of diagnosis of hemophagocytic lymphohistiocytosis in adults with
           lymphoid and myeloid malignancies
    • Authors: Egle Sumskiene; Ewa Pawłowicz; Cecilia Kämpe Björkvall; Maciej Machaczka
      Abstract: Publication date: Available online 24 July 2017
      Source:Acta Haematologica Polonica
      Author(s): Egle Sumskiene, Ewa Pawłowicz, Cecilia Kämpe Björkvall, Maciej Machaczka
      Background: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder of immune regulation. In patients over 60 years of age, HLH associated with hematological malignancies (hM-HLH) is the most prevalent. β-2-Microglobulin (B2M) plays an important role in antigen presentation and immunological regulation. Elevated B2M levels reflect T-cell activation. Objective: The aim of this study was to determine serum B2M concentrations in adults with hM-HLH and to interpret its significance in the context of overall survival (OS). Patients and methods: Serum B2M concentration was determined in 31 adults aged 22–84 years at the time of hM-HLH diagnosis. Lymphoid malignancy was diagnosed in 22 patients and myeloid malignancy in 9 patients. Results: The serum concentration of B2M was elevated in 100% of the examined patients. Mean and median serum B2M concentrations were 5.3 and 4.2mg/L, respectively (range 2–17mg/L). We have not found any significant differences in terms of the studied serum B2M concentrations between patients with T/NK-cell lymphomas, B-cell lymphomas, and myeloid malignancies. The outcome of HLH was poor in vast majority of patients with the median OS for the entire group of 46 days. Conclusions: Elevated serum B2M level is a frequent finding at the time of hM-HLH diagnosis in adults. It seems to be a useful indicator of HLH for its early detection and evaluation afterward, as well as for immediate therapeutic intervention. Further prospective studies answering the question whether serum B2M can be used as a prognostic factor in hM-HLH would be of interest.

      PubDate: 2017-08-01T10:30:42Z
      DOI: 10.1016/j.achaem.2017.07.001
  • Jak rozpoznawać i leczyć chorobę Gauchera: zarys patofizjologii,
           objawów klinicznych, metod diagnostycznych i leczenia
    • Authors: Egle Sumskiene; Maciej Machaczka
      Abstract: Publication date: Available online 24 July 2017
      Source:Acta Haematologica Polonica
      Author(s): Egle Sumskiene, Maciej Machaczka
      Rare diseases are a diagnostic challenge for modern medicine. Gaucher disease is a rare autosomal recessive lipid storage disorder caused by the deficient activity of the lysosomal enzyme glucocerebrosidase. In the absence of known affected family member, frequent symptoms of Gaucher disease, such as thrombocytopenia or splenomegaly, often lead to hematological diagnostic workup. This review highlights pathophysiology, signs and symptoms, diagnostic and therapeutic principles of Gaucher disease. Difficulties in diagnosis of Gaucher disease depends mainly on its rarity, but there is also the lack of awareness and limited knowledge about this disease. Basic knowledge of Gaucher disease should be familiar to all physicians, including hematologists.

      PubDate: 2017-08-01T10:30:42Z
      DOI: 10.1016/j.achaem.2017.07.003
  • Ryzyko przeniesienia czynników zakaźnych przez transfuzje w
    • Authors: Piotr Grabarczyk; Aneta Kopacz; Ewa Sulkowska; Aleksandra Kalińska
      Abstract: Publication date: Available online 24 July 2017
      Source:Acta Haematologica Polonica
      Author(s): Piotr Grabarczyk, Aneta Kopacz, Ewa Sulkowska, Aleksandra Kalińska
      Blood transfusion in Poland is the safest in history. High virological level of safety has been achieved mainly by improving not only the qualification of donors and methods used for donor screening, but also applying leukoreduction, pathogen reduction technology and grace period for serum. In this article, we discuss the improvement of the epidemic situation among blood donors for hepatitis B virus (HBV) and hepatitis C virus (HCV) and the increasing trend for HIV. Preliminary results of residual risk calculation for these pathogens are presented. Hepatitis E virus (HEV) and Babesia microti were considered as new factors potentially relevant for the safety of blood transfusion in our country. Due to evidence of West Nile virus (WNV) circulation in the ecosystem in Poland, it is also necessary to monitor the infections with this pathogen. In this article, it was emphasized that the reporting of all possible complications associated with transfusion and meticulous implementation of the look-back procedure play a key role for monitoring the risk of transmission of infectious agents by blood. It is especially important in view of the increasing epidemiological problems associated with emerging infectious agents.

      PubDate: 2017-08-01T10:30:42Z
      DOI: 10.1016/j.achaem.2017.07.006
  • Iksazomib u chorych z nawrotowym lub opornym na leczenie szpiczakiem
    • Authors: Krzysztof Giannopoulos; Wiesław Wiktor Jędrzejczak; Krzysztof Jamroziak; Dominik Dytfeld; Tadeusz Robak; Andrzej Hellmann
      Abstract: Publication date: Available online 23 July 2017
      Source:Acta Haematologica Polonica
      Author(s): Krzysztof Giannopoulos, Wiesław Wiktor Jędrzejczak, Krzysztof Jamroziak, Dominik Dytfeld, Tadeusz Robak, Andrzej Hellmann
      Ixazomib is a new agent registered in combination with lenalidomide and dexamethasone for the treatment of adult patients with multiple myeloma who have received at least one prior therapy. The drug is the first oral proteasome inhibitor. Registration data show improvement in progression-free survival time (20.6 vs 14.7 months, p=0.01) but recent analysis in Asian population also in overall survival (25.8 vs 15.8 months, HR=0.419, p=0.001) what placed with lack of ixazomib incremental toxicity may implicate clinical importance of the treatment in relapsed or refractory multiple myeloma. Oral dosing of the triplet regimen may decrease the number and duration of hospitalizations that allows for better social functioning and occupational performance, and thus impacts daily disease perception.

      PubDate: 2017-07-24T08:27:56Z
      DOI: 10.1016/j.achaem.2017.07.002
  • Efficacy and safety of bosutinib in the second and third line of treatment
           in chronic myeloid leukemia
    • Authors: Bogdan Ochrem; Tomasz Sacha
      Abstract: Publication date: Available online 10 July 2017
      Source:Acta Haematologica Polonica
      Author(s): Bogdan Ochrem, Tomasz Sacha

      PubDate: 2017-07-14T06:15:44Z
      DOI: 10.1016/j.achaem.2017.03.002
  • Możliwości leczenia indukcyjnego chorych na szpiczaka plazmocytowego
           kwalifikujących się do chemioterapii wysokodawkowanej wspomaganej
           autologiczną transplantacją komórek krwiotwórczych a aktualne
           zalecenia Polskiej Grupy Szpiczakowej
    • Authors: Agnieszka Druzd-Sitek; Jan Walewski
      Abstract: Publication date: Available online 15 June 2017
      Source:Acta Haematologica Polonica
      Author(s): Agnieszka Druzd-Sitek, Jan Walewski
      Despite significant progress in the treatment of multiple myeloma, it still remains an incurable disease. The introduction of new drugs has contributed to the increased overall survival of patients diagnosed with multiple myeloma and significantly improved their quality of life. Proteasome inhibitors, immunomodulators and monoclonal antibodies are often used in combination with chemotherapeutic agents. Despite the indisputable role of the new drugs for the treatment of myeloma, high-dose chemotherapy with autologous stem cell transplantation is still the method of choice in a group of younger patients without significant comorbidities. Induction treatment in myeloma patients eligible for autologous stem cell transplantation is particularly important, because the results of the first-line therapy impact further outcome of patients. The quality of the response to first-line treatment determines the progression-free survival. The choice of bortezomib, thalidomide, dexamethasone regimen as induction therapy, recommended by the Polish Myeloma Group, is in line with the recommendations of international experts. The results of the randomized clinical trials have demonstrated that the triple therapy, based on proteasom inhibitors, immunomodulators and steroids, is more effective than regimens involving two drugs only (proteasom inhibitors and steroids or immunomodulators and steroids). Sometimes, due to the clinical situation of the patient and/or unusual features of the disease, it is necessary to individualize induction therapy. The following article provides an overview of different induction regimens for myeloma patients in Poland and suggestions for modifications in justified cases.

      PubDate: 2017-06-17T21:18:01Z
      DOI: 10.1016/j.achaem.2017.06.001
  • Zalecenia Polskiej Grupy Szpiczakowej dotyczące rozpoznawania i leczenia
    • Authors: Anna Dmoszyńska; Lidia Usnarska-Zubkiewicz; Jan Walewski; Ewa Lech-Marańda; Adam Walter-Croneck; Barbara Pieńkowska-Grela; Grzegorz Charliński; Wiesław Wiktor Jędrzejczak; Bogdan Małkowski; Krzysztof Jamroziak; Agnieszka Druzd-Sitek; Dominik Dytfeld; Mieczysław Komarnicki; Tadeusz Robak; Artur Jurczyszyn; Joanna Mańko; Aleksander Skotnicki; Sebastian Giebel; Elżbieta Wiater; Ryszard Czepko; Janusz Meder; Krzysztof Giannopoulos
      Abstract: Publication date: Available online 7 June 2017
      Source:Acta Haematologica Polonica
      Author(s): Anna Dmoszyńska, Lidia Usnarska-Zubkiewicz, Jan Walewski, Ewa Lech-Marańda, Adam Walter-Croneck, Barbara Pieńkowska-Grela, Grzegorz Charliński, Wiesław Wiktor Jędrzejczak, Bogdan Małkowski, Krzysztof Jamroziak, Agnieszka Druzd-Sitek, Dominik Dytfeld, Mieczysław Komarnicki, Tadeusz Robak, Artur Jurczyszyn, Joanna Mańko, Aleksander Skotnicki, Sebastian Giebel, Elżbieta Wiater, Ryszard Czepko, Janusz Meder, Krzysztof Giannopoulos
      New drugs introduced in recent years to 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 the cornerstone for treatment of relapsed/refractory myeloma patients. Most patients profit from prolonged treatment composed of consolidation and maintenance treatment till progression. Besides the 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 therapeutic recommendation also for Waldenström macroglobulinemia and other plasmacytic dyscrasias.

      PubDate: 2017-06-12T19:08:31Z
      DOI: 10.1016/j.achaem.2017.05.003
  • Metastasis of solid tumors into bone marrow – Single center
    • Authors: Anna Kołda; Grzegorz Helbig; Anna Kopińska; Ryszard Wichary; Jacek Pająk; Sławomira Kyrcz-Krzemień
      Abstract: Publication date: Available online 31 May 2017
      Source:Acta Haematologica Polonica
      Author(s): Anna Kołda, Grzegorz Helbig, Anna Kopińska, Ryszard Wichary, Jacek Pająk, Sławomira Kyrcz-Krzemień
      Introduction: Metastases of solid tumors to the bone marrow are rarely reported. Clinical manifestation and laboratory findings remain uncharacteristic and lead to misdiagnosis. Detection of bone marrow metastases may have an impact on therapeutic decisions and is usually associated with poor prognosis. Aim: To characterize clinical picture and hematological findings in patients with bone marrow metastasis. Material and methods: We retrospectively reviewed medical records of patients with bone marrow metastases who were primary misdiagnosed with hematological malignancies. Results: Ten patients at median age of 51 years at diagnosis were included. There were following findings on admission: duopenia (n =7), pancytopenia (n =1), anemia (n =1) and skeletal lytic lesions (n =1). The diagnosis of prior cancer was reported in 3 patients and included multiple myeloma, breast cancer and oligoastrocytoma. Clinical manifestations were hepatomegaly (n =4), lymphadenopathy (n =4), skin pallor (n =3), cachexia (n =2) and hemorrhagic diathesis (n =2). Imaging studies revealed diffuse bone lesions (n =5), pulmonary infiltrates (n =2) and liver masses (n =2). Leukoerythroblastosis was demonstrated in 4 cases. Bone marrow aspirate detected the presence of abnormal cell population in 4 patients. In all studied patients a final diagnosis was established by immunohistochemistry of bone marrow biopsy. The following malignancies were detected: prostate adenocarcinoma (n =2), anaplastic microcellular carcinoma of unknown origin (n =2), adenocarcinoma of unknown origin (n =2), Ewing's sarcoma (n =1), breast cancer (n =1), clarocellular renal cancer (n =1) and neuroendocrine tumor (n =1). Nine out of the 10 metastatic patients died shortly after chemotherapy. Conclusions: Unexplained hematological abnormalities should arise the suspicion of bone marrow metastases.

      PubDate: 2017-06-02T15:41:08Z
      DOI: 10.1016/j.achaem.2017.05.001
  • Primary Hodgkin lymphoma of the nasopharynx: Case report
    • Authors: Justyna Grela-Kowalik; Dariusz Zatoński; Tomasz Zatoński
      Abstract: Publication date: Available online 25 May 2017
      Source:Acta Haematologica Polonica
      Author(s): Justyna Grela-Kowalik, Dariusz Zatoński, Tomasz Zatoński
      Primary extranodal location of Hodgkin lymphoma is uncommon. We present a case of a 43-year old man with isolated Hodgkin disease localized in the nasopharynx who presented with a complaint of bilateral nasal congestion. Primary location of Hodgkin lymphoma (HL) in the non-lymphoid tissue is very uncommon and the primary isolated involvement of the nasopharynx has been described in 35 cases in the worldwide literature.

      PubDate: 2017-05-28T10:26:37Z
      DOI: 10.1016/j.achaem.2017.04.001
  • Haemate P − zastosowanie w profilaktyce i leczeniu krwawień w chorobie
           von Willebranda oraz indukcji immunotolerancji w hemofilii A powikłanej
    • Authors: Andrzej Mital
      Abstract: Publication date: Available online 23 May 2017
      Source:Acta Haematologica Polonica
      Author(s): Andrzej Mital
      Haemate P is a plasma-derived concentrate used in the treatment of von Willebrand disease and haemophilia A. In comparison with other concentrates, this product is characterised by a high content of high-molecular-weight multimers. The ratio of von Willebrand factor (vWF:RCo) to factor VIII (FVIII) is 2.4:1. Haemate P is used with a high efficacy and safety when desmopressin (DDAVP) is ineffective or contraindicated, in all forms of von Willebrand disease (vWD), in the treatment of bleeding, in preparation for surgery and in prophylaxis in some patients, especially those with type 3 of the disease. The favourable vWF:RCo/FVIII ratio prevents accumulation of FVIII and related thromboembolic complications. Haemate P has been considered a gold standard in the treatment of von Willebrand disease for many years. This concentrate is used also in haemophilia A replacement therapy. One of the complications of haemophilia A treatment is the development of anti-FVIII antibodies called an inhibitor. The objective of the treatment of those patients is the elimination of the inhibitor, possibly through inducing immune tolerance (IT). The efficacy of Haemate P in immune tolerance induction both in the first and consecutive lines of treatment has been demonstrated by many reports. Replacement of highly-purified and recombinant FVIII concentrates in the case of failure to induce IT to concentrates with a high vWF:RCo content may be a beneficial treatment option in another attempt of inducing IT.

      PubDate: 2017-05-24T09:58:24Z
      DOI: 10.1016/j.achaem.2017.05.002
  • Metody oznaczania hemoglobiny płodowej
    • Authors: Elżbieta Górska; Anna Lemańska; Katarzyna Popko
      Abstract: Publication date: Available online 4 May 2017
      Source:Acta Haematologica Polonica
      Author(s): Elżbieta Górska, Anna Lemańska, Katarzyna Popko
      The identification of the new form of hemoglobin in fetus red blood cells (HbF), different from adult hemoglobin (HbA), was made over one hundred years ago. Since this time, various methods of fetal hemoglobin measurement have been designed. Most of them are based on the different biochemical characteristics of HbF. Fetal hemoglobin's affinity for oxygen is substantially greater than that of adult hemoglobin. The first techniques for the determination of fetal hemoglobin were based on its resistance to denaturation by alkaline solutions. Currently, the measurement of hemoglobin F is an important part of diagnosis of sickle cell disease, thalassemia, hereditary persistence of fetal hemoglobin, and fetomaternal hemorrhage. Amongst the most commonly used and clinically important methods, high-performance liquid chromatography, capillary electrophoresis, isoelectric focusing, capillary isoelectric focusing, Kleihauer–Betke test, and flow cytometry should be listed.

      PubDate: 2017-05-08T13:23:29Z
      DOI: 10.1016/j.achaem.2016.12.001
  • 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
  • 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
  • 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
  • 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
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