Myelofibrosis
Background
- Chronic myeloproliferative disorder
- Abnormal proliferation is seen in all 3 cell lines
- Typically in elderly
- Can be asymptomatic or symptomatic
- Can be primary or develop secondary to polycythemia vera or essential thrombocytosis
Clinical Features
- Palpable splenomegaly
- Bone pain
Differential Diagnosis
Evaluation
Workup
- CBC
- Blood smear
- Chem 7
- Bone marrow biopsy
Diagnosis[1]
- Anemia
- Pancytopenia (in late disease)
- Increased serum LDH
- Criteria: The diagnosis of primary myelofibrosis requires meeting all 3 major criteria and 2 minor criteria.
| Major criteria |
|---|
| Megakaryocyte proliferation and atypia with reticulin and/or collagen fibrosis,
or with increased marrow cellularity, granulocytic proliferation, and decreased erythropoiesis |
| Not meeting WHO criteria for CML, PV, MDS, or other myeloid neoplasm |
| Demonstration of JAK2V617F or other clonal marker or no evidence of reactive bone marrow fibrosis |
| Minor criteria |
|---|
| Leukoerythroblastosis |
| Increased serum LDH |
| Anemia |
| Palpable splenomegaly |
Management
- Blood transfusion
- Ruxolitinib
- Allogeneic stem cell transplantation
Also See
References
- Tefferi, A, et al. The 2008 World Health Organization classification system for myeloproliferative neoplasms: order out of chaos. Cancer. 2009; 115(17):3842-7,
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