Diagnosis and therapeutic management of a mast cell leukemia

https://www.islh.org/online-admin/posters/ISLH-418-0526091055.pdf

International Society for Laboratory Hematology

Joy Mouanes-Abelin, Corinne Ferrero-Vacher, Neila De Pooter, Danièle Aquaronne, Maria Hamoir, Alessandra Rosenthal, Thomas Cluzeau, Sami Benachour, Laetitia Riva, Pierre Toulon

• The Mast Cell Leukemia (MCL) is an extremely rare and aggressive subtype of systemic mastocytosis (SM) characterized by the infiltration of the bone marrow (BM) and/or other extra-cutaneous organs by clonal mast cells.

• The diagnosis of systemic Mastocytosis according to the ICC 2022 requires one major criterion, such as dense infiltrates of 15 or more mast cells in bone marrow biopsy (BMB) or other extracutaneous organs, and one minor criterion or at least three minor criteria, such as more than 25% atypical spindle-shaped mast cells, KIT mutation at codon 816, expression of CD2 and/or CD25 on mast cells or elevated serum tryptase levels above 20 ng/mL.

• Actually, the diagnosis of MCL requires the presence of at least 20% atypical mast cells in the BM or at least 10% in the blood.

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