Haema 2012; 3(1): 78-88
by Theodoros Marinakis, George Kanavos, Nikolaos I. Anagnostopoulos
Department of Clinical Hematology, General Hospital of Athens “G. Gennimatas”
Abstract
In the revised World Health Organization classification system for Chronic Myeloid Neo- plasms (2008) the diagnostic algorithms for Polycythemia Vera, Essential Thrombocythemia and Primary Myelofibrosis have been substantially changed to include informations regarding JAK2V617F and similar activating mutations, as well as pertinent histologic features of the bone marrow biopsy as diagnostic criteria. The Philadelphia negative Myeloproliferative Neoplasms category includes the three classic MPN: Polycythemia Vera, (PV), Essential Thrombocythemia (ET) and Primary Myelofibrosis (PMF), as well as Chronic Neutrophilic Leukemia, Chronic Eosinophilic leukemia not otherwise specified, Mastocytosis and Myeloproliferative neoplasm, unclassifiable. Patients with PV and ET should be defined as high risk if age is greater than 60 years or there is a history of previous thrombosis. High risk patients with PV should be managed with phlebotomy, low dose aspirin and cytoreduction, while high risk patients with ET should be managed with cytoreduction. For PMF several prognostic scoring systems have been proposed based on some adverse prognostic factors for survival including advanced age, marked anemia, leukocytosis, constitutional symptoms and presence of circulating blasts. Risk stratification in PMF should start with the International Prognostic Scoring System- IPSS for newly diagnosed patients and dynamic IPSS for patients during the disease course with the addition of cytogenetics evaluation and transfusion status. This prognostic assessment of patients with PMF may be useful for treatment decision-making.