Revised: February 8, 2014
Accepted: June 10, 2014
Published online: August 6, 2014
Myeloproliferative neoplasms include three diseases: polycythemia vera, essential thrombocythemia and primary myelofibrosis (PMF), currently diagnosed according to the 2008 World Health Organization criteria. Patients with PMF may encounter many complications, and, among these, disease progression is the most severe. Concerning prognostication of Myelofibrosis (MF), the International Prognostic scoring system (IPSS) (International Prognostic Scoring System) model at diagnosis and the Dynamic IPSS (DIPSS) anytime during the course of the disease may be useful to define survival of MF patients. The IPSS and the DIPSS are based on age greater than 65 years, presence of constitutional symptoms, hemoglobin level less than 10 g/dL, leukocyte count greater than 25 × 109/L, and circulating blast cells 1% or greater. Cytogenetic profile and mutational analysis seem to be the next step to implement MF prognostication. Concerning treatments, hydroxyurea has been considered until now the drug of choice when an anti-myeloproliferative effect is needed, but recent data on JAK inhibitors demonstrated a significant effect of these drugs on splenomegaly and symptoms.
Core tip: Myelofibrosis (MF) is a mutational/clinical-complex disease. Prognostication of MF is based on the International Prognostic scoring system (IPSS) model at diagnosis and on the Dynamic IPSS thereafter. Factors included in both models are: age > 65 years, constitutional symptoms, hemoglobin < 10 g/dL, leukocytes > 25 × 109/L, and circulating blast cells 1% or greater. Cytogenetic profile and mutational status help to better discriminate within each IPSS category. JAK inhibitors are new promising therapies with a molecular target, translating into a clinical benefit: spleen reduction MF-symptoms refief. Among JAK inhibitor, ruxolitinib has been approved for MF.