Acute mast cell leukemia

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Acute mast cell leukemia

Synonyms: Acute MCL

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.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.

Reference: Access aggregated data from Orphanet at Orphadata.

Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version April 2024

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