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Chronic myeloproliferative disorders (0)

The chronic myeloproliferative disorders are defined as clonal hematopoietic stem cell diseases in which there is overproduction of one or more of the formed elements of the blood in the absence of a definable cause. Hematopoiesis eventually extends from the bone marrow to other organs, such as the spleen and liver, and there is a variable rate of transformation to myelofibrosis or acute leukemia. Chronic myelogenous leukemia (CML) has long been the prototype for this group of illnesses. The discovery of the constitutively active fusion tyrosine kinase Bcr-Abl as the cause of the unregulated hematopoiesis in CML led to the development of effective targeted therapy in the form of the Abl tyrosine kinase inhibitors imatinib, dasatinib, and nilotinib. Until recently, however, therapy for the Bcr-Abl-negative chronic myeloproliferative disorders -- polycythemia vera (PV), idiopathic myelofibrosis (IMF), essential thrombocytosis (ET), and the hypereosinophilic syndrome (HES) -- was largely nonspecific and symptomatic.