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Chronic mast cell leukemia

MedGen UID:
1843226
Concept ID:
C5680130
Neoplastic Process
Synonyms: chronic mast cell leukemia; Chronic MCL
 
Monarch Initiative: MONDO:0035445
Orphanet: ORPHA566396

Definition

A rare form of mast cell leukemia characterized by the presence of at least 20% mast cells in bone marrow aspirate smears but often mature mast cell morphology, low proliferation rate, and absence of organ damage and C findings (cytopenias, hepatomegaly, ascites, portal hypertension, splenomegaly, skeletal lesions, malabsorption). The disease course is less aggressive than in the acute form, although patients may later progress. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChronic mast cell leukemia

Professional guidelines

PubMed

Pullarkat ST, Wu W, Pullarkat V
Cancer Treat Res 2021;181:167-178. doi: 10.1007/978-3-030-78311-2_10. PMID: 34626361
Horny HP, Sotlar K, Reiter A, Valent P
Expert Rev Hematol 2014 Aug;7(4):431-7. doi: 10.1586/17474086.2014.942280. PMID: 25025369
Tefferi A, Elliott MA, Pardanani A
Mayo Clin Proc 2006 Apr;81(4):553-63. doi: 10.4065/81.4.553. PMID: 16610578

Recent clinical studies

Diagnosis

Valent P, Berger J, Cerny-Reiterer S, Peter B, Eisenwort G, Hoermann G, Müllauer L, Mannhalter C, Steurer M, Bettelheim P, Horny HP, Arock M
Ann Hematol 2015 Feb;94(2):223-31. Epub 2014 Sep 11 doi: 10.1007/s00277-014-2207-9. PMID: 25209843Free PMC Article

Prognosis

Valent P, Sotlar K, Sperr WR, Reiter A, Arock M, Horny HP
Leuk Res 2015 Jan;39(1):1-5. Epub 2014 Sep 30 doi: 10.1016/j.leukres.2014.09.010. PMID: 25443885Free PMC Article
Valent P, Berger J, Cerny-Reiterer S, Peter B, Eisenwort G, Hoermann G, Müllauer L, Mannhalter C, Steurer M, Bettelheim P, Horny HP, Arock M
Ann Hematol 2015 Feb;94(2):223-31. Epub 2014 Sep 11 doi: 10.1007/s00277-014-2207-9. PMID: 25209843Free PMC Article

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