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Annual Clinical Updates in Hematological Malignancies: a continuing medical education series: polycythemia vera and essential thrombocythemia: 2011 update on diagnosis, risk-stratification, and management.

Abstract：

DISEASE OVERVIEW:Polycythemia vera (PV) and essential thrombocythemia (ET) are myeloproliferative neoplasms primarily characterized by erythrocytosis and thrombocytosis, respectively. Other disease features include leukocytosis, splenomegaly, thrombohemorrhagic complications, vasomotor disturbances, pruritus, and a small risk of disease progression into acute leukemia or myelofibrosis. DIAGNOSIS:Diagnosis is based on JAK2 mutation status (PV and ET), serum erythropoietin (Epo) level (PV), and bone marrow histopathology (ET). The presence of a JAK2 mutation and subnormal serum Epo level confirm a diagnosis of PV. Differential diagnosis in ET should include chronic myelogenous leukemia and prefibrotic myelofibrosis. RISK STRATIFICATION:Current risk stratification in PV and ET is designed to estimate the likelihood of thrombotic complications: high-risk-age > 60 years or presence of thrombosis history; low-risk-absence of both of these two risk factors. Presence of extreme thrombocytosis (platelet count > 1,000 x 10⁹/L) might be associated with acquired von Willebrand syndrome (AvWS) and, therefore, risk of bleeding. Risk factors for shortened survival in both PV and ET include age > 60 years, leukocytosis, history of thrombosis, and anemia. RISK-ADAPTED THERAPY:Survival is near-normal in ET and reasonably long in PV. The 10-year risk of leukemic/fibrotic transformation is < 1%/1% in ET and < 5%/10% in PV. In contrast, the risk of thrombosis exceeds 20%. The main goal of therapy is therefore to prevent thrombohemorrhagic complications and this is effectively and safely accomplished by the use of low-dose aspirin (PV and ET), phlebotomy (PV), and hydroxyurea (high risk PV and ET). Treatment with busulfan or interferon-a is usually effective in hydroxyurea failures.

journal_name

Am J Hematol

journal_title

American journal of hematology

authors

Tefferi A

doi

10.1002/ajh.21946

subject

Has Abstract

pub_date

2011-03-01 00:00:00

pages

292-301

issue

eissn

0361-8609

issn

1096-8652

journal_volume

pub_type

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Annual Clinical Updates in Hematological Malignancies: a continuing medical education series: polycythemia vera and essential thrombocythemia: 2011 update on diagnosis, risk-stratification, and management.