Hairy cell leukemia variant

Orpha code: 300878OMIM code:

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.

Disease data
Classification

Disease

Synonyms
HCL-v
HCL-v
Leukemic reticuloendotheliosis variant
Prolimfocytarny wariant białaczki włochatokomórkowej
Prolimfocytarny wariant HCL
Leukemic reticuloendotheliosis variant
Prolymphocytic variant of HCL
Prolymphocytic variant of hairy cell leukemia
ORPHA code
300878
OMIM code
-
ICD10 code
C91.4
ICD11 code
-

No additional description.

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