myelofibrosis
A myeloproliferative neoplasm characterized by bone marrow fibrosis, extramedullary hematopoiesis, and progressive bone marrow failure.
Full Definition
Myelofibrosis is a clonal hematopoietic stem cell disorder classified as a myeloproliferative neoplasm. The disease is characterized by progressive bone marrow fibrosis, which leads to cytopenias and compensatory extramedullary hematopoiesis, typically manifesting as splenomegaly and hepatomegaly. Myelofibrosis can occur as a primary disorder (primary myelofibrosis) or secondary to other myeloproliferative neoplasms such as polycythemia vera or essential thrombocythemia. The disease is associated with constitutional symptoms and has a variable prognosis. Allogeneic stem cell transplantation remains the only curative therapy, while JAK inhibitors can provide symptomatic relief.
Usage
Usage note: Specify 'primary' or 'secondary' myelofibrosis when the distinction is clinically relevant.
In Context
- "The patient's progressive anemia and massive splenomegaly were consistent with myelofibrosis." — clinical assessment
- "Bone marrow biopsy showed grade 3 fibrosis confirming the diagnosis of myelofibrosis." — pathology report