Primary myelofibrosis
ORPHA:824· ICD-10 D47.4
Definition
A rare myeloproliferative neoplasm characterized by stem-cell derived clonal over proliferation of mature myeloid lineages, such as erythrocytes, leukocytes, and megakaryocytes, with variable degrees of megakaryocyte atypia, associated with reticulin and/or collagen bone marrow fibrosis, osteosclerosis, ineffective erythropoiesis, angiogenesis, extramedullary hematopoiesis, and abnormal cytokine expression.
- Prevalence
- 1-9 / 100 000
- Inheritance
- Not applicable
- Age of onset
- Adult