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- W2023784583 abstract "Abstract Mast cell leukemia (MCL) is a very rare form of aggressive systemic mastocytosis accounting for < 1% of all mastocytosis. It may appear de novo or secondary to previous mastocytosis and shares more clinicopathologic aspects with systemic mastocytosis than with acute myeloid leukemia. Symptoms of mast cell activation—involvement of the liver, spleen, peritoneum, bones, and marrow—are frequent. Diagnosis is based on the presence of ≥ 20% atypical mast cells in the marrow or ≥ 10% in the blood; however, an aleukemic variant is frequently encountered in which the number of circulating mast cells is < 10%. The common phenotypic features of pathologic mast cells encountered in most forms of mastocytosis are unreliable in MCL. Unexpectedly, non-KIT D816V mutations are frequent and therefore, complete gene sequencing is necessary. Therapy usually fails and the median survival time is < 6 months. The role of combination therapies and bone marrow transplantation needs further investigation." @default.
- W2023784583 created "2016-06-24" @default.
- W2023784583 creator A5000705579 @default.
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- W2023784583 creator A5067808997 @default.
- W2023784583 creator A5088509972 @default.
- W2023784583 date "2013-02-21" @default.
- W2023784583 modified "2023-10-17" @default.
- W2023784583 title "Mast cell leukemia" @default.
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- W2023784583 doi "https://doi.org/10.1182/blood-2012-07-442400" @default.
- W2023784583 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/23243287" @default.
- W2023784583 hasPublicationYear "2013" @default.
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