Myelofibrosis Signs and Symptoms
Definition
- Clonal myeloproliferative stem cell disorder characterized by reactive bone marrow fibrosis, extramedullary hematopoiesis, and abnormal cytokine expression leading to systemic symptoms
- Belongs to a group of heterogeneous disorders of the hematopoietic system which is collectively known as Philadelphia chromosome-negative myeloproliferative neoplasms (MPN)
- Present either as a de novo disorder [primary myelofibrosis (PMF)] or can develop from the transformation of polycythemia vera (PV) and essential thrombocythemia (ET)
- PMF is characterized by clonal proliferation of early hematopoietic stem cells
- Often accompanied by Janus kinase 2 (JAK2), calreticulin (CALR) or myeloproliferative leukemia (MPL) gene mutations
- Sub-classified into prefibrotic or early stage PMF and overt fibrotic-stage PMF
- Occurs mainly in middle aged and older adults
- Median age of onset is 67 years
- Most common presenting symptom is fatigue and other symptoms include enlarged spleen, weight loss, low grade fever, night sweats, bone pain
Signs and Symptoms
- Constitutional symptoms include fatigue, fever, weight loss and night sweats
- Severe anemia, bone pain, splenic infarct, pruritus, thrombosis, bleeding, cachexia
- Approximately 30% are asymptomatic