Classic hairy cell leukemia

Orpha code: 58017OMIM code:

Definition

A rare, slowly progressive, chronic leukemia characterized by presence of abnormal B-lymphocytes (medium sized with abundant irregular pale cytoplasm, hair-like cytoplasmic projections/ruffled cytoplasmic border, a round or bean-shaped nucleus and absent nucleoli) in the blood or bone marrow, spleen and peripheral blood pancytopenia, notable monocytopenia, and marked susceptibility to infection. The characteristic immunophenotype is CD11c+, CD25+, CD103+ and CD123+ with a BRAF mutation in most cases.

Disease data
Classification

Disease

Synonyms
HCL-C
HCL
Leukemic reticuloendotheliosis
Leukemic reticuloendotheliosis
ORPHA code
58017
OMIM code
-
ICD10 code
C91.4
ICD11 code
2A82.2

No additional description.

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