Acute mast cell leukemia: A rare but highly aggressive hematopoietic neoplasm

Costopoulos, Myrto; Uzunov, Madalina; Bories, Dominique; Charlotte, Frédéric; Maloum, Karim; Arock, Michel

doi:10.1002/dc.23965

Cited by 5 publications

(5 citation statements)

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“…CD25 is more useful than CD2 which is less constantly expressed [17] . MCL shows an immunophenotypic reactivity similar to the other types of SM, however, a third of MCL cases have been noted to have a double negative CD2/CD25 immunophenotype [2] . A variable proportion of MCL cases may express CD30 [18] .…”

Section: Discussionmentioning

confidence: 94%

“…The main symptomatology includes, but is not limited to, hypotension, rash, pruritus, diarrhea, hyperacidity, musculoskeletal pain, tachycardia, and fever. SM is diagnosed by demonstrating mast cell infiltration in at least one extracutaneous organ, with the bone marrow being the most commonly involved organ and the most commonly used to establish diagnosis [2] . Its diagnosis requires fulfilling the major diagnostic criterion and at least one of the four minor criteria, or three minor criteria.…”

Section: Introductionmentioning

confidence: 99%

“…SM is subdivided into five main variants based on the mast cell burden (“B” findings) and the presence/absence of organ impairment secondary to mast cell infiltration (“C” findings) ( Table 1 ) [ 4 , 5 ]. Among the different variants of SM, MCL is considered the rarest occurring in less than 1% of SM cases and carries the worst prognosis [ 2 , 6 ]. By definition, MCL must meet the World Health Organization (WHO) 2017 criteria for SM and is marked by diffuse bone marrow infiltration of immature or atypical mast cells accounting for at least 20% of all nucleated cells in the bone marrow (BM) aspirate smear and at least 10% in the peripheral blood smear (PBS).…”

Section: Introductionmentioning

confidence: 99%

“…Progression from aleukemic MCL to leukemic MCL has been reported to occur [12] . Moreover, MCL is divided between an acute variant (67–93% of cases) and the less common chronic variant based on the presence or absence of “C” findings [ 2 , 7 , 13 , 14 ]. The latter has been associated with a more indolent course at disease outset, but overall similar prognosis [ 7 , 8 , 14 ].…”

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Acute aleukemic mast cell leukemia: Report of a case and review of the literature

Galura

Cherukuri

Hakim

et al. 2020

Leukemia Research Reports

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Section: Discussionmentioning

confidence: 94%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Acute aleukemic mast cell leukemia: Report of a case and review of the literature

Galura

Cherukuri

Hakim

et al. 2020

Leukemia Research Reports

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“…Because of the rarity of MCL, accounting for 1% of adult SM, our current knowledge of the disease, including its clinicopathologic features, molecular characteristics, prognosis and treatment, is mainly restricted to case reports and only limited case series [ 6 , 25 , 26 , 27 ].…”

Section: Mcl: Defining Criteria Leukemic and Aleukemic Variants Prima...mentioning

confidence: 99%

Mast Cell Leukemia: An Update with a Practical Review

Zanelli

Quintini

Magnasco

et al. 2023

Cancers

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Mast cell leukemia (MCL) is the leukemic form of SM with at least 20% mostly immature mast cells on bone marrow aspirate. MCL may develop de novo, in the absence of a prior SM, or it may represent a progression from a previous SM. MCL may be sub-divided into the more frequent, aggressive acute form with signs of organ damage (C-findings) and the chronic form lacking C-findings and presenting a more stable course, although over time, progression to acute MCL is common. The 2022 WHO subtype of MCL with an associated hematological neoplasm was renamed MCL with an associated myeloid neoplasm in the 2022 International Consensus Classification (ICC). The relevance of the distinction between the leukemic and aleukemic forms based on the percentage of circulating mast cells is a matter of debate. The current knowledge on MCL is restricted mainly to single reports or case series with a limited number of larger studies. Our aim is to provide a comprehensive overview of this rare disease in terms of clinical manifestations, morphology, phenotype, molecular characteristics, differential diagnosis, outcome and treatment. A general overview on mastocytosis is also included.

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