Mast cell leukemia
From Wikipedia, the free encyclopedia
| Acute mast cell leukemia Classification and external resources |
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| ICD-O: | M9742/3 |
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| MeSH | D007946 |
Mast cell leukemia is a subtype of acute myeloid leukemia that usually occurs de novo but can, rarely, evolve from transformation of chronic myeloid leukemia into the more aggressive acute myeloid leukemia. In a small proportion of cases, acute mast cell leukemia may evolve from a more progressive form of systemic mastocytosis. The diagnosis of acute mast cell leukemia by the WHO criteria includes the requirement for a prevalence of 20% neoplastic mast cells in marrow and 10% in blood.
[edit] Clinical course
Acute mast cell leukemia is a rapidly progressive disorder with leukemic mast cells in blood and in large numbers in marrow. The common signs and symptoms include fever, headache, flushing of face and trunk. Symptoms include abdominal pain, bone pain, and peptic ulcer which are more prevalent than in other subtypes of acute myeloid leukemia. These former symptoms are due to relase of a subtance called histamine from neoplastic mast cells. Enlargement of the liver and spleen, or hepatosplenomegaly is characteristic. The mast cells release also a chemical substance called heparin which can lead to serious bleeding.
[edit] Prognosis
Acute mast cell leukemia is extremly aggressive and has a grave prognosis. Medial survival after diagnosis is only about 3 months.
[edit] References
Lichtman MA and Segel GB, "Uncommon phenotypes of acute myelogenous leukemia: basophilic, mast cell, eosinophilic, and myeloid dendritic cell subtypes: a review."; Blood Cells Mol Dis. 2005 Nov-Dec;35(3):370-83.
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