Primary myelofibrosis: risk stratification and treatment

Abstract

Objective: The purpose of this preentation was to overview the risk-adapted therapy for PMF patients in era of novel agents. Presentation summary. Primary myelofibrosis (PMF) is one of myeloproliferative neoplasms according to 2008 WHO classification. The median survival of patients with PMF is 5-7 years, nevertheless the low risk patients survive twice longer. Risk assessment for survival in patients with primary myelofibrosis is established using International Scoring System (IPSS), dynamic-IPSS (DIPSS), age adjusted DIPSS and DIPSS-PLUS. IPSS uses 5 risk factors at the time of diagnosis age > 65years, constitution symptoms, hemoglobin < 100 g/l, WBC count > 25x 109/l and circulating blasts>1%. DIPSS is used to estimate survival during disease course DIPSS-plus incorporates additional 3 independent prognostic factors: need for red cell transfusion, thrombocytopenia <100 x 109/l and unfavorable karyotype. [...].