Mast cell leukemiaC94.3
Synonym(s)
Mast cell leukemia; Mast cell reticulosis
DefinitionThis section has been translated automatically.
Mastocytosis with systemic malignant mast cell proliferation and leaching of atypical mast cells into the peripheral blood
ManifestationThis section has been translated automatically.
Especially patients with systemic mastocytosis without cutaneous skin involvement are at risk (approx. 30%).
Clinical featuresThis section has been translated automatically.
Corresponding to mastocytosis.
DiagnosisThis section has been translated automatically.
>20% mast cells in the bone marrow aspirate, mast cells usually immature, often blastar. >10% mast cells in blood smear.
TherapyThis section has been translated automatically.
Both aggressive systemic mastocytosis (ASM) and mast cell leukemia require cytoreductive therapy because they are life-threatening.
Cytostatic drugs such as cyclophosphamide, chlorambucil and others can be tried. However, the chances of success are low.
Progression/forecastThis section has been translated automatically.
Infaust, usually rapidly progressive course. In mastocytosis with skin involvement malignancy development is very rare.
LiteratureThis section has been translated automatically.
- Arber DA et al (2016) The 2016 revision to the World Health Organization classification of myeloidneoplasms
and acute leukemia. Blood 127:2391-405.
- Torrey E et al (1990) Malignant mastocytosis with circulating mast cells. Am J Hematol 34: 283-286