Homepage > Rare diseases > Search

Search for a rare disease

*
(*) mandatory field

Acute mast cell leukemia

Suggest an update
Your message has been sent Your message has not been sent. Please contact an administrator.
Disease definition

A rare systemic mastocytosis characterized by the presence of at least 20% usually immature and atypical mast cells in bone marrow aspirate smears. In classic mast cell leukemia, mast cells account for at least 10% of peripheral white blood cells, although the aleukemic variant with less than 10% mast cells is more common. C-findings (cytopenias, hepatomegaly, ascites, portal hypertension, splenomegaly, skeletal lesions, malabsorption), indicative of organ damage due to mast cell infiltration, are usually present at diagnosis, while skin lesions are absent in most cases. Prognosis is generally poor.

ORPHA:566393

Classification level: Subtype of disorder

Synonym(s):
  • Acute MCL

Source: PubMed ID 28254862

Prevalence: -

Inheritance: Not applicable

Age of onset:

ICD-10: C94.3

ICD-11: 2A21.00

UMLS: C5680128

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)
The documents contained in this website are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.