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Chronic mast cell leukemia

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Disease 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.

ORPHA:566396

Classification level: Subtype of disorder

Synonym(s):
  • Chronic MCL

Source: PubMed ID 28254862

Prevalence: -

Inheritance: Not applicable

Age of onset:

ICD-10: C94.3

ICD-11: 2A21.00

UMLS: C5680130

A summary on this disease is available in Français, Español, Deutsch, Italiano, Nederlands
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

Guidelines
Emergency guidelines
Français (2024.pdf) - Orphanet Urgences
Clinical practice guidelines
Diagnostic Keys
Français (2024) - Les clés du diagnostic Logo FSMR
Patient-Centered Outcome Measures (PCOMs)
Access questionnaires assessing quality of life in this disease (English)
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