Are we altering the natural history of primary myelofibrosis?

Abstract

Primary myelofibrosis (PMF) is a clonal hematologic malignancy with a variable disease course; survival ranges from months to years. Historically, only allogeneic hematopoietic stem cell transplantation (alloHSCT) has demonstrated an ability to alter the natural history of PMF, but high treatment-related mortality risks limit the utility of alloHSCT to a minority of patients with PMF or myelofibrosis secondary to other myeloproliferative neoplasms. The recent development of therapies that regulate the Janus kinase-signal transducer and activator of transcription signaling pathway has changed the treatment landscape from primarily palliative treatment to potential disease modification.

Keywords: JAK inhibitors; Myelofibrosis; Proinflammatory cytokines; Spleen volume; Targeted therapy.