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TheScientificWorldJOURNAL
Volume 11 (2011), Pages 1131-1137
doi:10.1100/tsw.2011.108
Short Review

Defining the Thrombotic Risk in Patients with Myeloproliferative Neoplasms

1Hematology and Clinical Immunology Unit, Thoracic, and Vascular Sciences Department, Padova University School of Medicine, Padova, Italy
2Cardiac, Thoracic, and Vascular Sciences Department, Padova University School of Medicine, Padova, Italy
3Division of Immunohematology and Transfusion Medicine, Ospedali Riuniti di Bergamo, Italy

Received 23 January 2011; Revised 12 May 2011; Accepted 13 May 2011

Academic Editor: Edward J. Benz

Copyright © 2011 Fabrizio Vianello et al.

Abstract

Polycythemia vera (PV) and essential thrombocythemia (ET) are two Philadelphia-negative myeloproliferative neoplasms (MPN) associated with an acquired mutation in the JAK2 tyrosine kinase gene. There is a rare incidence of progression to myelofibrosis and myeloid metaplasia in both disorders, which may or may not precede transformation to acute myeloid leukemia, but thrombosis is the main cause of morbidity and mortality. The pathophysiology of thrombosis in patients with MPN is complex. Traditionally, abnormalities of platelet number and function have been claimed as the main players, but increased dynamic interactions between platelets, leukocytes, and the endothelium do probably represent a fundamental interplay in generating a thrombophilic state. In addition, endothelial dysfunction, a well-known risk factor for vascular disease, may play a role in the thrombotic risk of patients with PV and ET. The identification of plasma markers translating the hemostatic imbalance in patients with PV and ET would be extremely helpful in order to define the subgroup of patients with a significant clinical risk of thrombosis.