465 research outputs found

    Interview with Salah Taher

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    في هذه المقابلة، يتحدث صلاح طاهر، رائد الفن التجريدي في مصر، عن لقائه بالأديب المصري عباس محمود العقاد. أجرى المقابلة حسن شمس الدين.In this interview, Salah Taher, a pioneer of abstract art in Egypt, recounts meeting Egyptian author Abbas Mahmoud al-Akkad. The interview was conducted by Hassan Shams al-Din

    Management of Non-Transfusion-Dependent Thalassemia: A Practical Guide

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    Despite their transfusion-independence, non-transfusion-dependent thalassemia (NTDT) patients experience a variety of serious clinical complications that require prompt and comprehensive management. Transfusion therapy may still be an important part of management of this disease, in cases of acute stress, to support growth and development in childhood, or to prevent clinical morbidities stemming from ineffective erythropoiesis or hemolytic anemia. Although splenectomy is associated with improvements in hemoglobin levels, it leads to several short- and long-term adverse events, warranting caution in application of this intervention. Fetal hemoglobin induction therapy has been evaluated in non-randomized studies, with benefits extending beyond hematologic improvements to lowering morbidity risk. Effective and safe iron chelation therapy is now available for NTDT patients in whom iron overload develops, irrespective of transfusions, due to increased intestinal absorption, ultimately leading to clinically high iron burden levels and subsequent morbidity. 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    Thrombosis in thalassemia: Why are we so concerned?

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    Although life expectancy of thalassemia patients has markedly improved over the last few decades, patients still suffer from many complications of this congenital disease. The presence of a high incidence of thromboembolic events (TEE), mainly in β-thalassemia intermedia (β-TI), has led to the identification of a hypercoagulable state in these patients. In this review, the molecular and cellular mechanisms leading to hypercoagulability in thalassemia are highlighted, with a special focus on thalassemia intermedia being the group with the highest incidence of thrombotic events as compared to other types of thalassemia. Clinical experience and available clues on optimal management are also discussed. © 2011 Informa Healthcare USA, Inc.Atichartakarn V, 2002, BRIT J HAEMATOL, V118, P893, DOI 10.1046-j.1365-2141.2002.03711.x; Atichartakarn V, 2003, INT J HEMATOL, V77, P299, DOI 10.1007-BF02983790; Yashar Vered Borenstain-Ben, 1993, American Journal of Hematology, V44, P63, DOI 10.1002-ajh.2830440114; Pignatti CB, 1998, ACTA HAEMATOL-BASEL, V99, P76; Borgna-Pignatti C, 2004, HAEMATOLOGICA, V89, P1187; BUTTHEP P, 1995, THROMB HAEMOSTASIS, V74, P1045; Butthep P, 1997, SE ASIAN J TROP M S3, V28, P141; Cadili A, 2008, AM J MED, V121, P371, DOI 10.1016-j.amjmed.2008.02.014; Cappellini MD, 2010, ANN N Y ACAD SCI, V1202, P231; Cappellini MD, 2000, BRIT J HAEMATOL, V111, P467, DOI 10.1046-j.1365-2141.2000.02376.x; Cappellini MD, 2005, ANN N Y ACAD SCI, V1054, P317; Crary SE, 2009, BLOOD, V114, P2861, DOI 10.1182-blood-2009-04-210112; DELPRINCIPE D, 1993, BRIT J HAEMATOL, V84, P111; ELDOR A, 1991, BLOOD, V77, P1749; ELDOR A, 1989, AM J HEMATOL, V32, P94, DOI 10.1002-ajh.2830320204; Habib A, 2008, HAEMATOL-HEMATOL J, V93, P941, DOI 10.3324-haematol.12460; Helley D, 1996, THROMB HAEMOSTASIS, V76, P322; HERSHKO C, 1978, BRIT J HAEMATOL, V40, P255, DOI 10.1111-j.1365-2141.1978.tb03662.x; Hovav T, 1999, BRIT J HAEMATOL, V106, P178; Iolascon A, 2001, HAEMATOLOGICA, V86, P1112; Karimi M, 2010, THROMB HAEMOSTASIS, V103, P989, DOI 10.1160-TH09-09-0661; Kuypers FA, 2004, CELL MOL BIOL, V50, P147; Manfre L, 1999, AM J ROENTGENOL, V173, P1477; Ruf A, 1997, BRIT J HAEMATOL, V98, P51, DOI 10.1046-j.1365-2141.1997.1502965.x; Rund D, 2005, NEW ENGL J MED, V353, P1135, DOI 10.1056-NEJMra050436; Steinberg MH, 2009, DISORDERS OF HEMOGLOBIN: GENETICS, PATHOPHYSIOLOGY, AND CLINICAL MANAGEMENT, 2ND EDITION, P1, DOI 10.1017-CBO9780511596582; Sumiyoshi A, 1992, SE ASIAN J TROP M S2, V23, P29; Taher A, 2006, THROMB HAEMOSTASIS, V96, P488, DOI 10.1160-TH06-05-0267; Taher A, 2009, BRIT J HAEMATOL, V147, P634, DOI 10.1111-j.1365-2141.2009.07848.x; Taher AT, 2008, BLOOD REV, V22, P283, DOI 10.1016-j.blre.2008.04.001; Taher AT, 2010, BRIT J HAEMATOL, V150, P486, DOI 10.1111-j.1365-2141.2010.08220.x; Taher AT, 2011, BRIT J HAEMATOL, V152, P512, DOI 10.1111-j.1365-2141.2010.08486.x; Taher AT, 2010, J THROMB HAEMOST, V8, P2152, DOI 10.1111-j.1538-7836.2010.03940.x; Taher AT, 2010, J THROMB HAEMOST, V8, P54, DOI 10.1111-j.1538-7836.2009.03651.x; Taher AT, 2010, BLOOD, V115, P1886, DOI 10.1182-blood-2009-09-243154; Tavazzi D, 2001, BRIT J HAEMATOL, V112, P48, DOI 10.1046-j.1365-2141.2001.02482.x; Tripodi A, 2009, HAEMATOL-HEMATOL J, V94, P1520, DOI 10.3324-haematol.2009.010546; WINICHAGOON P, 1981, Southeast Asian Journal of Tropical Medicine and Public Health, V12, P556; Zalloua PA, 2003, THROMB HAEMOSTASIS, V89, P767; ZURLO MG, 1989, LANCET, V2, P2716191

    Stroke in thalassemia: A dilemma

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    [No abstract available]KARIMI M, 2007, AM J HEMATOL 0815; Taher A, 2006, THROMB HAEMOSTASIS, V96, P488, DOI 10.1160-TH06-05-026796

    Iron overload in β-thalassemia intermedia: An emerging concern

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    Purpose of Review: The aim is to overview recent evidence on consequences, assessment, and management of iron overload in transfusion-independent patients with β-thalassemia intermedia. Recent Findings: Despite their transfusion-independence, β-thalassemia intermedia patients can still accumulate iron due to increased intestinal absorption. Recent observational studies show that iron burden in this group of patients can ultimately reach considerably high thresholds, and leads to a variety of serious morbidities involving the liver, endocrine glands, and arguably the vascular system. The diagnosis of iron overload in this patient population can follow established guidelines from β-thalassemia major patients, although with careful interpretation of spot serum ferritin levels. Data from a recent randomized clinical trial demonstrated the efficacy and safety of iron chelation therapy in decreasing liver iron concentration in this group of patients, which may ultimately help in reducing morbidity risk. Summary: Iron overload in transfusion-independent patients with β-thalassemia intermedia deserves careful attention, and prompt diagnosis and management are recommended. © 2013 Wolters Kluwer Health | Lippincott Williams and Wilkins.Casanovas G, 2013, HAEMATOLOGICA, V98, P444, DOI 10.3324-haematol.2012.069807; Gardenghi S, 2010, J CLIN INVEST, V120, P4466, DOI 10.1172-JCI41717; Ginzburg Y, 2011, BLOOD, V118, P4321, DOI 10.1182-blood-2011-03-283614; Karimi M, 2011, EUR J INTERN MED, V22, P607, DOI 10.1016-j.ejim.2011.05.013; Li HH, 2010, NAT MED, V16, P177, DOI 10.1038-nm.2073; Maakaron JE, 2013, ANN HEPATOL, V12, P142; Musallam KM, 2012, ANN HEMATOL, V91, P235, DOI 10.1007-s00277-011-1291-3; Musallam KM, 2011, HEMOGLOBIN, V35, P503, DOI 10.3109-03630269.2011.605499; Musallam KM, 2012, BLOOD CELL MOL DIS, V49, P136, DOI 10.1016-j.bcmd.2012.06.001; Musallam KM, 2011, HAEMATOL-HEMATOL J, V96, P1605, DOI 10.3324-haematol.2011.047852; Musallam KM, 2012, CSH PERSPECT MED, V2, DOI 10.1101-cshperspect.a013482; Musallam KM, 2012, BLOOD, V120; Musallam KM, 2012, BLOOD, V120; Musallam KM, 2012, BLOOD REV, V26, pS16, DOI 10.1016-S0268-960X(12)70006-1; Musallam KM, 2011, EUR J HAEMATOL, V87, P539, DOI 10.1111-j.1600-0609.2011.01706.x; Musallam KM, 2012, THROMB RES, V130, P695, DOI 10.1016-j.thromres.2012.07.013; Nai A, 2012, BLOOD, V119, P5021, DOI 10.1182-blood-2012-01-401885; Origa R, 2007, HAEMATOL-HEMATOL J, V92, P583, DOI 10.3324-haematol.10842; Pakbaz Z, 2007, PEDIATR BLOOD CANCER, V49, P329, DOI 10.1002-pbc.21275; Parrow NL, 2012, BLOOD, V119, P3187, DOI 10.1182-blood-2012-01-405563; Rivella S, 2012, BLOOD REV, V26, pS12, DOI 10.1016-S0268-960X(12)70005-X; Taher A, 2008, HAEMATOL-HEMATOL J, V93, P1584, DOI 10.3324-haematol.13098; Taher A, 2009, BRIT J HAEMATOL, V146, P569, DOI 10.1111-j.1365-2141.2009.07810.x; Taher A, 2012, HAEMATOLOGICA, V96, P0924; Taher A., 2012, HAEMATOLOGICA, V96; Taher AT, 2010, AM J HEMATOL, V85, P288, DOI 10.1002-ajh.21626; Taher AT, 2010, BRIT J HAEMATOL, V150, P486, DOI 10.1111-j.1365-2141.2010.08220.x; Taher AT, 2012, BLOOD, V120, P970, DOI 10.1182-blood-2012-02-412692; Taher AT, 2011, BRIT J HAEMATOL, V152, P512, DOI 10.1111-j.1365-2141.2010.08486.x; Taher AT, 2012, BLOOD, V120, P3258; Taher AT, 2010, BLOOD, V115, P1886, DOI 10.1182-blood-2009-09-243154; Wood John C, 2011, Hematology Am Soc Hematol Educ Program, V2011, P443, DOI 10.1182-asheducation-2011.1.443; Ziyadeh FN, 2012, NEPHRON CLIN PRACT, V121, pC136, DOI 10.1159-00033978789

    Covert brain ischaemia in splenectomised adults with thalassemia intermedia: An emerging entity

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    Contemporary approaches to treatment of beta-thalassemia intermedia

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    Beta-thalassemia intermedia (TI) is associated with a variety of serious clinical complications that require proactive and comprehensive management. These include skeletal deformities and osteopenia, compensatory extramedullary hematopoiesis and tumor formation, progressive splenomegaly, a hypercoagulable state resulting in thromboembolic events and pulmonary hypertension, and increased gastrointestinal iron absorption that often results in nontransfusional iron overload and liver damage. Although TI is generally considered a non-transfusion-dependent thalassemia, transfusion therapy may be an important part of the comprehensive management of this disease. This review describes the current state of the art for medical management of TI, with particular focus on the roles of splenectomy, transfusion, and iron chelation therapy. © 2012 Elsevier Ltd.Aessopos A, 2005, ANN NY ACAD SCI, V1054, P342, DOI 10.1196-annals.1345.041; Aessopos A, 2007, HAEMATOL-HEMATOL J, V92, P658, DOI 10.3324-haematol.10915; Aessopos A, 2001, BLOOD, V97, P3411, DOI 10.1182-blood.V97.11.3411; Amoozgar H, 2011, EUR J HAEMATOL, V87, P61, DOI 10.1111-j.1600-0609.2011.01620.x; [Anonymous], FERR SUMM PROD CHAR; [Anonymous], 2010, EXJADE PACK INS; [Anonymous], EXJADE SUMM PROD CHA; Ataga KI, 2007, BRIT J HAEMATOL, V139, P3, DOI 10.1111-j.1365-2141.2007.06740.x; Atichartakarn V, 2003, INT J HEMATOL, V78, P139, DOI 10.1007-BF02983382; Bisharat N, 2001, J INFECTION, V43, P182, DOI 10.1053-jinf.2001.0904; Borgna-Pignatti C, 2010, ANN NY ACAD SCI, V1202, P214, DOI 10.1111-j.1749-6632.2010.05550.x; Borgna-Pignatti C, 2007, BRIT J HAEMATOL, V138, P291, DOI 10.1111-j.1365-2141.2007.06654.x; Cappellini MD, 2005, SEMIN HEMATOL, V42, pS19, DOI 10.1053-j.seminhematol.2005.01.001; Cappellini MD, 2000, BRIT J HAEMATOL, V111, P467, DOI 10.1046-j.1365-2141.2000.02376.x; COSSU P, 1981, EUR J PEDIATR, V137, P267, DOI 10.1007-BF00443255; Davies JM, 2011, BRIT J HAEMATOL, V155, P308, DOI 10.1111-j.1365-2141.2011.08843.x; Eder AF, 2007, ARCH PATHOL LAB MED, V131, P708; Gardenghi S, 2010, J CLIN INVEST, V120, P4466, DOI 10.1172-JCI41717; Haidar R, 2010, EUR SPINE J, V19, P871, DOI 10.1007-s00586-010-1357-2; Karimi M, 2010, EUR J HAEMATOL, V84, P52, DOI 10.1111-j.1600-0609.2009.01356.x; Karimi M, 2011, EUR J INTERN MED, V22, P607, DOI 10.1016-j.ejim.2011.05.013; Karimi M, 2010, PEDIATR HEMAT ONCOL, V27, P205, DOI 10.3109-08880011003639952; Kontoghiorghes GJ, 2010, HEMOGLOBIN, V34, P251, DOI 10.3109-03630269.2010.486335; Ladis V, 2010, BRIT J HAEMATOL, V151, P504, DOI 10.1111-j.1365-2141.2010.08346.x; Li HH, 2010, NAT MED, V16, P177, DOI 10.1038-nm.2073; Musallam KM, 2011, EUR J HAEMATOL, V87, P73, DOI 10.1111-j.1600-0609.2011.01623.x; Musallam KM, 2011, HAEMATOL-HEMATOL J, V96, P1605, DOI 10.3324-haematol.2011.047852; Novartis Pharmaceutical Corporation, 2010, DESF PACK INS; Porter J, 2011, 16 C EUR HEM ASS JUN; Porter JB, 2001, SEMIN HEMATOL, V38, P63, DOI 10.1053-shem.2001.20145; Rigano P, 2010, BRIT J HAEMATOL, V151, P509, DOI 10.1111-j.1365-2141.2010.08397.x; Sankaran VG, 2010, NEW ENGL J MED, V363, P2258, DOI 10.1056-NEJMcibr1010767; STURGEON P, 1955, BRIT J HAEMATOL, V1, P264, DOI 10.1111-j.1365-2141.1955.tb05509.x; Taher A, 2008, HAEMATOL-HEMATOL J, V93, P1584, DOI 10.3324-haematol.13098; Taher A, 2006, THROMB HAEMOSTASIS, V96, P488, DOI 10.1160-TH06-05-0267; Taher A, 2006, BLOOD CELL MOL DIS, V37, P12, DOI 10.1016-j.bcmd.2006.04.005; Taher A, 2009, BRIT J HAEMATOL, V147, P634, DOI 10.1111-j.1365-2141.2009.07848.x; Taher A, 2002, HEMOGLOBIN, V26, P107, DOI 10.1081-HEM-120005447; Taher AT, 2008, BLOOD REV, V22, P283, DOI 10.1016-j.blre.2008.04.001; Taher AT, 2010, BRIT J HAEMATOL, V150, P486, DOI 10.1111-j.1365-2141.2010.08220.x; Taher AT, 2011, BRIT J HAEMATOL, V152, P512, DOI 10.1111-j.1365-2141.2010.08486.x; Taher AT, 2010, J THROMB HAEMOST, V8, P54, DOI 10.1111-j.1538-7836.2009.03651.x; Taher AT, 2010, BLOOD, V115, P1886, DOI 10.1182-blood-2009-09-243154; Taher ATPJ, 2011, 53 AM SOC HEM ANN M; Treadwell MJ, 2001, SEMIN HEMATOL S1, V38, P77; Voskaridou E, 2010, BRIT J HAEMATOL, V148, P332, DOI 10.1111-j.1365-2141.2009.07930.x9151

    Iron chelation therapy for non-transfusion-dependent thalassemia (NTDT): A status quo

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    [No abstract available]Alkrawinthawong K., 2011, HEMATOLOGY, V16, P113; Chan JCW, 2006, BRIT J HAEMATOL, V133, P198, DOI 10.1111-j.1365-2141.2006.05984.x; COSSU P, 1981, EUR J PEDIATR, V137, P267, DOI 10.1007-BF00443255; Ladis V, 2010, BRIT J HAEMATOL, V151, P504, DOI 10.1111-j.1365-2141.2010.08346.x; Musallam K., 2013, HAEMATOLOGICA, V98, P486; Musallam KM, 2013, CURR OPIN HEMATOL, V20, P187, DOI 10.1097-MOH.0b013e32835f5a5c; Musallam KM, 2013, BLOOD CELL MOL DIS, V51, P35, DOI 10.1016-j.bcmd.2013.01.015; Musallam KM, 2011, HAEMATOL-HEMATOL J, V96, P1605, DOI 10.3324-haematol.2011.047852; Musallam KM, 2012, BLOOD REV, V26, pS16, DOI 10.1016-S0268-960X(12)70006-1; Musallam KM, 2013, HAEMATOLOGICA, V98, P833, DOI 10.3324-haematol.2012.066845; OLIVIERI NF, 1992, BLOOD, V79, P2741; Pippard M J, 1988, Birth Defects Orig Artic Ser, V23, P29; Pootrakul P, 2003, BRIT J HAEMATOL, V122, P305, DOI 10.1046-j.1365-2141.2003.04412.x; Rombos Y, 2000, HAEMATOLOGICA, V85, P115; Taher A., 2013, HAEMATOLOGICA, V98, P165; Taher A., 2012, HAEMATOLOGICA, V96; Taher A, 2013, HAEMATOLOGICA, V98, P486; Taher A. T., 2013, GUIDELINES MANAGEMEN; Taher AT, 2013, ANN HEMATOL, V92, P1485, DOI 10.1007-s00277-013-1808-z; Taher AT, 2010, BRIT J HAEMATOL, V150, P486, DOI 10.1111-j.1365-2141.2010.08220.x; Taher AT, 2012, BLOOD, V120, P970, DOI 10.1182-blood-2012-02-412692; Taher AT, 2013, AM J HEMATOL, V88, P409, DOI 10.1002-ajh.23405; Taher AT, 2013, AM J HEMATOL, V88, P503, DOI 10.1002-ajh.23445; Voskaridou E, 2010, BRIT J HAEMATOL, V148, P332, DOI 10.1111-j.1365-2141.2009.07930.x11

    Deferasirox for the treatment of iron overload in non-transfusion-dependent thalassemia

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    Non-transfusion-dependent thalassemia (NTDT) defines a group of patients who do not require regular transfusions for survival, but are at significant risk of iron accumulation from underlying disease-related mechanisms distinct from transfusional iron overload. Management of iron overload in NTDT has received little attention compared with that of β-thalassemia major, despite evidence of significant iron-induced complications with advancing age. The efficacy and safety of the iron chelator deferasirox in NTDT has been evaluated in two pilot studies and the first prospective, randomized, placebo-controlled study (THALASSA) of any chelator in NTDT. Treatment with deferasirox for up to 2 years yielded a sustained reduction in iron burden, with a clinically manageable safety profile. Following these trial data, deferasirox is the first iron chelator approved for use in NTDT patients, and with NTDT guidelines now available, physicians are better equipped to achieve effective monitoring and management of iron burden in NTDT. © 2013 Informa UK Ltd.Akrawinthawong K, 2011, HEMATOLOGY, V16, P113, DOI 10.1179-102453311X12940641877768; [Anonymous], 2013, EXJADE DEF US PRESCR; [Anonymous], 2013, GUID MAN NONTR DEP T; [Anonymous], 2011, FERR SUMM PROD CHAR; [Anonymous], 2010, DESFERAL DEFEROXAMIN; Apotex, 2011, FERR PRESCR INF; Au WY, 2009, ANN HEMATOL, V88, P947, DOI 10.1007-s00277-009-0701-2; Aydinok Y, 2013, HAEMATOLOGICA S1, V98; Bruin GJM, 2008, DRUG METAB DISPOS, V36, P2523, DOI 10.1124-dmd.108.022962; Calabro A, 2006, HAEMATOLOGICA S1, V91; CAMASCHELLA C, 1995, HAEMATOLOGICA, V80, P58; Cao A, 2010, GENET MED, V12, P61, DOI 10.1097-GIM.0b013e3181cd68ed; Cappellini MD, 2009, ANNU REV MED, V60, P25, DOI 10.1146-annurev.med.60.041807.123243; Cappellini MD, 2010, HAEMATOL-HEMATOL J, V95, P557, DOI 10.3324-haematol.2009.014696; 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    Evaluation of the 5mg-g liver iron concentration threshold and its association with morbidity in patients with β-thalassemia intermedia

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    Iron overload may still occur in transfusion-independent patients with β-thalassemia intermedia due to increased intestinal iron absorption. In this study, we evaluated the association between iron overload, using a liver iron concentration threshold of therapeutic significance (≥. 5. mg-g), and morbidity in 168 chelation naive patients with β-thalassemia intermedia. We demonstrated that patients with a liver iron concentration ≥. 5. mg-g have a significantly higher prevalence of several serious vascular and endocrine-bone morbidities than do patients with . 5. mg-g, and we established absolute morbidity risk values differentiating both groups. We also demonstrated that the association between iron overload and morbidity in such patients is independent of the effects of advancing age and disease severity. These findings suggest that treating iron burden in β-thalassemia intermedia may be associated with reduction in serious morbidity risk. © 2013 Elsevier Inc.Belhoul KM, 2012, ANN HEMATOL, V91, P1107, DOI 10.1007-s00277-012-1412-7; Haidar R, 2011, BONE, V48, P425, DOI 10.1016-j.bone.2010.10.173; Kell DB, 2009, BMC MED GENOMICS, V2, DOI 10.1186-1755-8794-2-2; Musallam KM, 2011, HEMOGLOBIN, V35, P503, DOI 10.3109-03630269.2011.605499; Musallam KM, 2012, BLOOD CELL MOL DIS, V49, P136, DOI 10.1016-j.bcmd.2012.06.001; Musallam KM, 2011, HAEMATOL-HEMATOL J, V96, P1605, DOI 10.3324-haematol.2011.047852; Musallam KM, 2012, BLOOD REV, V26, pS16, DOI 10.1016-S0268-960X(12)70006-1; Musallam KM, 2012, THROMB RES, V130, P695, DOI 10.1016-j.thromres.2012.07.013; Pootrakul P, 2003, BRIT J HAEMATOL, V122, P305, DOI 10.1046-j.1365-2141.2003.04412.x; Rivella S, 2012, BLOOD REV, V26, pS12, DOI 10.1016-S0268-960X(12)70005-X; St Pierre TG, 2005, BLOOD, V105, P855, DOI 10.1182-blood-2004-01-0177; Taher A, 2008, HAEMATOL-HEMATOL J, V93, P1584, DOI 10.3324-haematol.13098; Taher AT, 2012, BLOOD, V120, P970, DOI 10.1182-blood-2012-02-412692; Taher AT, 2010, BLOOD, V115, P1886, DOI 10.1182-blood-2009-09-243154; Wood JC, 2005, BLOOD, V106, P1460, DOI 10.1182-blood-2004-10-39825101
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