Лечение агрессивного системного мастоцитоза и тучноклеточного лейкоза. Обзор литературы и серия клинических наблюдений Treatment of Aggressive Systemic Mastocytosis and Mast Cell Leukemia. Literature Review and Case Reports

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.