Hairy cell leukemia variant
ORPHA:300878· ICD-10 C91.4
Definition
A rare, malignant splenic B-cell lymphoma/leukemia characterized by circulating abnormal lymphocytes with intermediate morphology between prolymphocytes and hairy cells with positive expression of CD11c and negative expression of CD25, CD123 and the BRAFV600E mutation. Manifestations include splenomegaly, elevated white blood cell (WBC) count, hyper-cellular bone marrow and anemia/thrombocytopenia, but no monocytopenia.
- Prevalence
- Unknown
- Inheritance
- Unknown
- Age of onset
- Adult, Elderly