Myelofibrosis (MF) is a rare blood cancer where dysregulation of the JAK-STAT pathway drives the proliferation of abnormal blood stem cells, local inflammation and fibrosis in the bone marrow. These changes compromise marrow function and lead to a chronic inflammatory state, driving the three hallmarks of MF:
- progressive anemia, often in association with thrombocytopenia or other cytopenias.
- constitutional symptoms such as fatigue, night sweats, fever, bone pain and pruritus.
- enlargement of the spleen (splenomegaly), which can be associated with abdominal distension, pain and early satiety.
The median survival for all patients with MF is about 6 years but it is considerably worse for high-risk patients at just 2.25 years, respectively. Transfusion dependency and moderate to severe anemia are critical negative prognostic factors in MF and inversely correlated with quality of life.