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lüll New advances in the pathogenesis and therapy of essential thrombocythemia Levine RL; Heaney MHematology Am Soc Hematol Educ Program 2008[]; ä (ä): 76-82Essential thrombocythemia (ET) is a hematopoietic disorder that manifests clinically as thrombocytosis, and patients with ET are at increased risk for developing thrombosis, myelofibrosis, and transformation to acute myeloid leukemia. Although ET was recognized as a distinct clinical syndrome more than 6 decades ago and was classified as a myeloproliferative neoplasm (MPN) by William Dameshek in 1951, the molecular pathogenesis of ET remained unknown until 2005, when activating mutations in the JAK2 tyrosine kinase (JAK2V617F) were identified in a significant proportion of patients with ET, polycythemia vera (PV) and primary myelofibrosis (PMF). In addition, subsequent studies have identified gain-of-function mutations in the thrombopoietin receptor (MPL) in a subset of patients with JAK2V617F-negative ET, suggesting that JAK2 activation by distinct mechanisms contributes to the pathogenesis of ET. Despite these important observations, important questions remain regarding the role of JAK2/MPL mutations in ET pathogenesis, the etiology of JAK2/MPL negative ET, the factors that distinguish ET from other MPNs with the JAK2V617F mutation, and the role of JAK2-targeted therapies for the treatment of these MPNs.|Amino Acid Substitution[MESH]|Blood Platelets/enzymology[MESH]|Chromosomes, Human, Pair 9[MESH]|Codon/genetics[MESH]|Erythropoiesis/genetics[MESH]|Glucosephosphate Dehydrogenase/genetics[MESH]|Granulocytes/enzymology[MESH]|Homozygote[MESH]|Humans[MESH]|Janus Kinase 2/genetics[MESH]|Leukemia, Myeloid, Acute/enzymology/*etiology/genetics/*therapy[MESH]|Mutation[MESH]|Receptors, Thrombopoietin/genetics[MESH]|Thrombocythemia, Essential/enzymology/*etiology/genetics/*therapy[MESH]|Thrombocytosis/etiology/therapy[MESH]|Thrombopoiesis/genetics[MESH]|Uniparental Disomy/genetics[MESH] |