Vol. 17 No. 2 (2024), MYELOID TUMORS
Vol. 17 No. 2 (2024)
MYELOID TUMORS

Treatment of Aggressive Systemic Mastocytosis and Mast Cell Leukemia. Literature Review and Case Reports

Published 01.04.2024
Vsevolod Gennadevich Potapenko
City Clinical Hospital No. 31, 3 Dinamo pr-t, Saint Petersburg, Russian Federation, 197110
E.V. Morozova
RM Gorbacheva Scientific Research Institute of Pediatric Oncology, Hematology and Transplantation; IP Pavlov First Saint Petersburg State Medical University, 6/8 L’va Tolstogo ul., Saint Petersburg, Russian Federation, 197022 3 National Center for Clinical Morphological Diagnosis, 32 Slavy pr-t, Saint Petersburg, Russian Federation, 192071
PDF_2024-17-2_180-194 (Russian)

Keywords

mastocytoses
tryptase
mast cells
mast cell leukemia
KIT gene
cladribine
imatinib

How to Cite

1.
Potapenko V.G., Morozova E.V. Treatment of Aggressive Systemic Mastocytosis and Mast Cell Leukemia. Literature Review and Case Reports. Клиническая онкогематология. 2024;17(2):180-194. doi:10.21320/2500-2139-2024-17-2-180-194
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Abstract

Aim. To provide case reports on the course and treatment of aggressive systemic mastocytosis (ASM) and mast cell leukemia (MCL).

Materials & Methods. This paper reports the histories of adult patients with ASM (n = 4) and MCL (n = 2) treated with cytoreduction predominantly at the City Clinical Hospital No. 31 (Saint Petersburg). The patients were 36–61 years of age (median 50 years); there were 4 women and 2 men.

Results. All patients showed stable response to cladribine (n = 3), imatinib (n = 2), and combined lenalidomide, elotuzumab, and dexamethasone treatment with subsequent allogeneic hematopoietic stem cell transplantation (n = 1). With a median follow-up of 124 months (range 55–186 months), 1 out of 2 MCL patients died due to the concomitant hypereosinophilic syndrome progression and secondary infectious complication.

Conclusion. Cladribine therapy in 2 ASM patients and imatinib therapy in 2 MCL patients yielded stable long-term response with good quality of life being maintained. More comparative studies are required for efficacy assessment and optimum sequencing of chemotherapeutic drugs.

PDF_2024-17-2_180-194 (Russian)

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