How to determine the prognosis and survival of myeloproliferative neoplasms

There is no cure for myeloproliferative neoplasms, and the goals of treatment are to control peripheral blood counts, adequately control disease-related symptoms, reduce the risk of thrombosis-hemorrhage, and delay progression to leukemia. Neither PV nor ET prognostic score includes symptoms: International Prognostic Scoring System for ET (IPSET): based on white blood cell count ≥ 11 × 109/L (1 point); age ≥ 60 years (2 points); history of thrombosis (1 point) is classified as low risk 0, intermediate risk 1-2 points with median survival of 24.5 years; high risk 3-4 points with median survival of 13.8 years. PV International Working Group on Myeloproliferative Tumors Research and Treatment (IWG-MRT): based on white blood cell count ≥ 15 × 109/L (1 point); deep vein thrombosis (1 point); age 57-66 years (2 points) and age ≥ 67 years (5 points); classified as low risk 0 points, median survival 26 years, intermediate risk 1-2 points, median survival 15 years; high risk ≥ 3 points, median survival 8.3 years. Only MF focused on symptoms, MF International Prognostic Score System (DIPSS): age, hemoglobin, leukocytes, peripheral blood primitive cells, systemic symptoms. Score low-risk OS not reached; intermediate-risk-1 14.2 years; intermediate-risk-2 4 years; high-risk 1.5 years.