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treatment of primary myelofibrosis (PMF)🚧 施工中
treatment of primary myelofibrosis (PMF)
(Am J Hematol 2021;96:145)
• In absence of adverse prognostic factors (eg, anemia or sx) → no treatment
• Allogeneic HSCT only potential cure → consider in young Pts w/ high-risk disease
• Supportive care: transfusions; ESA if Epo <500 (risk ↑ splenomegaly); consider androgens vs. immunomodulatory agents (eg, lenalidomide) + prednisone; hydroxyurea; ? splenectomy if refractory to transfusions, failed chemoRx, painful splenomegaly
JAK inh:
- ruxo-LI-tinib (JAK1/2) ↓ sx, ↓ splenomegaly, ↑ survival; preferred (NEJM 2012;366:787 & 799);
- fedratinib (JAK2; JAMA Oncol2015;1:643)
- pacritinib & momelotinib under study
Median survival
- ~6 y (JCO 2012;30:2981)
- transformation into AML occurs at a rate of ~8%/y