hematology and oncology
Primary Myelofibrosis (PMF)
Prognosis for Primary Myelofibrosis
The median survival in primary myelofibrosis is 5 years from onset, but variation is wide; some patients have a rapidly progressing disorder, including development of Acute Myeloid Leukemia (AML) , with short survival, but most have a more indolent course. Only Unfavorable prognostic markers include hemoglobin<10 g/dL (<100 g/L), a history of transfusions, leukocytosis, and a platelet count<100,000/mcL (<1000×109/L). Patients in the least favorable risk group usually survive<1 year, but those with low-risk disease have a median survival of 10 years. A number of useful risk stratification systems are available to aid prognosis and guide decisions to institute medical therapy or stem cell transplantation. The Dynamic International Prognostic Scoring System (DIPSS) for Primary Myelofibrosis can be used to predict progression or survival as the disease evolves ( Prognosis references ). Some scoring systems, such as (genetically inspired prognostic scoring system [ Validated symptom assessment tools (eg, Myelofibrosis Symptom Assessment Form) are also available for patients with myelofibrosis and can be useful to monitor therapeutic responses ( Prognosis references ). Patients with polycythemia vera or essential thrombocythemia with myelofibrosis usually have a much better prognosis than those with primary myelofibrosis, and their outcome can be predicted by using the MYelofibrosis SECondary to PV and ET Prognostic Model (MYSEC-PM— Prognosis references ). 1.Passamonti F, Cervantes F, Vannuchi AM, et al: Dynamic International Prognostic Scoring System (DIPSS) predicts progression to acute myeloid leukemia in primary myelofibrosis.Blood115:1703–1709, 2010. 2.Tefferi A, Guglielmelli P, Nicolosi M, et al: GIPSS: genetically inspired prognostic scoring system for primary myelofibrosis.Leukemia32(7):1631–1642, 2018. doi:10.1038/s41375-018-0107-z 3.Tefferi A, Guglielmelli P, Lasho TL, et al: MIPSS70+ Version 2.0: Mutation and Karyotype-Enhanced International Prognostic Scoring System for Primary Myelofibrosis.J Clin Oncol36(17):1769–1770, 2018. doi:10.1200/JCO.2018.78.9867 4.Tefferi A: Primary myelofibrosis: 2023 update on diagnosis, risk-stratification, and management.Am J Hematol98(5):801–821, 2023. doi:10.1002/ajh.26857 5.Mesa RA, Schwager S, Radia D, et al: The Myelofibrosis Symptom Assessment Form (MFSAF): an evidence-based brief inventory to measure quality of life and symptomatic response to treatment in myelofibrosis.Leuk Res33(9):1199–1203, 2009. doi:10.1016/j.leukres.2009.01.035 6.Passamonti F, Giorgino T, Mora B, et al: A clinical-molecular prognostic model to predict survival in patients with post polycythemia vera and post essential thrombocythemia myelofibrosis.Leukemia31(12):2726–2731, 2017. doi: 10.1038/leu.2017.169 