Abstract
The myeloproliferative syndromes encompass four major clinical entities: chronic myelogenous leukemia (CML), polycythemia vera (PV), essential thrombocythemia (ET), and idiopathic myelofibrosis (IM). These disorders are clonal myeloid stem cell disorders characterized by the autonomous proliferation of the clone, which results in the overproduction of one or more hematopoietic cell lineages. The clinical presentation and natural history of each disorder is distinct, but the features of the disorders often overlap (e.g., CML with myelo-fibrosis or PV with thrombocytosis).
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Carella AM, Lerma E, Corretti MT, et al. Autografting with Ph-mobilized hematopoietic progenitor cells in chronic myelogenous leukemia. Blood 1999;93:1534–1539.
Hansen JA, Gooley TA, Martin PJ, et al. Bone marrow transplants from unrelated donors for patients with chronic myelogenous leukemia. N Engl J Med 1998;338:962–968.
Hughes TP, Kaeda J, Branford S, et al. Frequency of major molecular responses to imatinib or interferon alfa plus cytarabine in newly diagnosed chronic myeloid leukemia. N Engl J Med 2003;349:1423–1432.
Kantarjian H, Talpaz M, O’Brien S, et al. High-dose imatinib therapy in newly diagnosed Philadelphia chromosome-positive chronic phase chronic myeloid leukemia. Blood 2004; 103:2873–2878.
Landolfi R, Marchioli R, Kutti J, et al. Efficacy and safety of low-dose aspirin in polycythemiavera. N Engl J Med 2004;350:114–124.
Lee SJ, Anasetti C, Horowitz MH, et al. Initial therapy for chronic myelogenous leukemia: playing the odds. J Clin Oncol 1998; 16:2897–2903.
Levine RL, Wadleigh M, Cools J, et al. Activating mutation in the tyrosine kinase JAK2 in polycythemia vera, essential thrombocythemia, and myeloid metaplasia with myelofibrosis. Cancer Cell 2005;7:387–397.
Moliterno AR, Hankins D, Spivak JL. Impaired expression of the thrombopoietin receptor by platelets from patients with polycythernia vera. N Engl J Med 1998;338:572–580.
Nimer SD. Essential thrombocythernia: another “heterogeneous disease” better understood? Blood 1999;93:415–416.
O’Brien SG, Guilhot F, Larson RA, et al. Imatinib compared with interferon and low-dose cytarabine for newly diagnosed chronic-phase chronic myeloid leukemia. N Engl J Med 2003;348:994–1004.
Prchal JT. Polycythemia vera and other primary polycythemias. Curr Opin Hematol 2005;12:112–116.
Shah NP, Tran C, Lee FY, et al. Overriding imatinib resistance with a novel ABL kinase inhibitor. Science 2005;305:399–401.
Talpaz M, Shah N, Kantarjian H, et al. Activity of the ABL kinase inhibitor desatinib in imatinib-resistant Philadelphia chromosome positive leukemias. New Eng J Med 2006;354:2531–2541.
Tefferi A. Myelofibrosis: update on pathogenesis and treatment. In: Hematology 2004: American Society of Hematology Education Program Book. Washington, DC: American Society of Hematology, 2004:151–153.
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© 2007 Humana Press Inc., Totowa, NJ
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Paquette, R., Hiller, E., Munker, R. (2007). The Myeloproliferative Syndromes. In: Munker, R., Hiller, E., Glass, J., Paquette, R. (eds) Modern Hematology. Contemporary Hematology. Humana Press. https://doi.org/10.1007/978-1-59745-149-9_8
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DOI: https://doi.org/10.1007/978-1-59745-149-9_8
Publisher Name: Humana Press
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