Vol 12, Supp. C (2021)
Case report
Published online: 2021-06-11

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Treatment-resistant aggressive systemic mastocytosis

Karolina Chromik1, Paulina Lis2, Patryk Węglarz1, Grzegorz Helbig1

Abstract

Systemic mastocytosis (SM) is a rare neoplastic disease of the haematopoietic system caused by an excessive proliferation of pathological mast cells which accumulate in various organs and lead to multi-organ damage. The course of the disease may be chronic, requiring only symptomatic treatment, or aggressive, when cytoreductive therapy is required.

The case study describes a 79-year-old male with advanced aggressive systemic mastocytosis. The patient initially received 2 cycles of cladribine, but due to disease progression, midostaurin was given as a second line of the treatment. The starting dose of the drug was reduced due to cytopenia. Additionally, severe gastrointestinal side effects were observed. Despite the treatment, the patient’s general condition deteriorated, he was constantly dependent on blood transfusion. Later, he developed multiple organ failure and eventually died.

Aggressive systemic mastocytosis may initially present at advanced disease stage and be associated with resistance to treatment. The lack of therapeutic effect after cladribine and midostaurin is associated with very poor prognosis and short survival.

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Hematology in Clinical Practice