Chronic mast cell leukemia

Orpha code: 566396OMIM code:

Definition

A rare form of mast cell leukemia characterized by the presence of at least 20% mast cells in bone marrow aspirate smears but often mature mast cell morphology, low proliferation rate, and absence of organ damage and C findings (cytopenias, hepatomegaly, ascites, portal hypertension, splenomegaly, skeletal lesions, malabsorption). The disease course is less aggressive than in the acute form, although patients may later progress.

Disease data
Classification

Clinical subtype

Synonyms
Chronic MCL
Chronic MCL
ORPHA code
566396
OMIM code
-
ICD10 code
C94.3
ICD11 code
-

No additional description.

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