Abstract | PURPOSE OF REVIEW: RECENT FINDINGS: Several studies have reported on the occurrence of JAK2(V617F) in approximately 50% of patients with essential thrombocythemia and its presence has been associated with advanced age at diagnosis, higher hemoglobin and leukocyte levels, and increased rate of polycythemic transformation. In contrast, the mutation did not appear to affect the incidence of thrombotic, leukemic, or fibrotic events. There is increasing evidence regarding the thrombogenic role of neutrophils in essential thrombocythemia and this might partly explain the superior overall performance by hydroxyurea, compared with anagrelide, in a recent randomized study. SUMMARY: Although it is in vogue to consider essential thrombocythemia as more than one disease in terms of both molecular phenotype (presence or absence of JAK2(V617F)) and putative pattern of myelopoiesis (monoclonal versus polyclonal), it is yet to be shown that such differences influence either the natural history of the disease or current therapy. From a treatment standpoint, hydroxyurea is now confirmed to be the drug of choice for high-risk patients with essential thrombocythemia.
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Authors | Ayalew Tefferi |
Journal | Current opinion in hematology
(Curr Opin Hematol)
Vol. 13
Issue 2
Pg. 93-8
(Mar 2006)
ISSN: 1065-6251 [Print] United States |
PMID | 16456375
(Publication Type: Journal Article, Review)
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Chemical References |
- Antineoplastic Agents
- Platelet Aggregation Inhibitors
- JAK2 protein, human
- Janus Kinase 2
- Aspirin
- Hydroxyurea
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Topics |
- Antineoplastic Agents
(therapeutic use)
- Aspirin
(therapeutic use)
- Bone Marrow Examination
(methods)
- Humans
- Hydroxyurea
(therapeutic use)
- In Situ Hybridization, Fluorescence
- Janus Kinase 2
(genetics)
- Platelet Aggregation Inhibitors
(therapeutic use)
- Point Mutation
(genetics)
- Thrombocythemia, Essential
(diagnosis, drug therapy, genetics)
- Thrombosis
(etiology)
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