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Review
. 2023 Mar 8;15(6):1664.
doi: 10.3390/cancers15061664.

Mast Cell Leukemia: An Update with a Practical Review

Magda Zanelli  1 , Martina Quintini  2 , Salvatore Magnasco  3 , Lara Aprile  4 , Andrea Palicelli  1 , Maurizio Zizzo  5 , Francesca Sanguedolce  6 , Stefano Ricci  1 , Saverio Pancetti  7   8 , Valeria Zuccalà  9 , Veronica Martino  1 , Giuseppe Broggi  10 , Rosario Caltabiano  10 , Alberto Cavazza  1 , Paola Parente  11 , Cristina Mecucci  2 , Giovanni Martino  2   12 , Stefano Ascani  12
Affiliations
Review

Mast Cell Leukemia: An Update with a Practical Review

Magda Zanelli et al. Cancers (Basel). .

Abstract

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.

Keywords: KIT mutation; MCL-AHN; MCL-AMN; mast cell leukemia; midostaurin; systemic mastocytosis.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
High power view of BM biopsy diffusely infiltrated by promastocytes (hematoxylin and eosin, magnification 300×; original image from Prof. S.A.).
Figure 2
Figure 2
High-power view of BM biopsy diffusely infiltrated by atypical cells with pleomorphic nuclei (within green circles); in the background, numerous eosinophils were found (red arrow) (hematoxylin and eosin, magnification 400×; original image from Prof. S.A.
Figure 3
Figure 3
Medium power view of BM infiltrate highlighted by diffuse and intense CD117 positivity (CD117 immunostaining, magnification 100×; original image from Prof. S.A.).
Figure 4
Figure 4
High-power view of BM infiltrate with diffuse and intense tryptase positivity (Tryptase immunostaining, magnification 400×; original image from Prof. S.A.).
Figure 5
Figure 5
High-power view of BM infiltrate showing a diffuse and intense CD25 positivity (CD25 immunostaining, magnification 400×; original image from Prof. S.A.).
See this image and copyright information in PMC

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