Systemic mastocytosis with recurrent anaphylactic shock and multiple organ dysfunction failure.

Background: Systemic mastocytosis (SM) is a rare neoplasm. The symptoms of this disease vary among patients. The authors describe a rare case of SM with recurrent anaphylactic shock and multiple organ dysfunction failure.

Methods: Hematologic investigation, bone marrow aspirate and biopsy, and cytogenetic analysis were performed.

Results: The patient was rescued with positive treatment, administered on prednisolone and H1/H2-receptor blocking agents. Corticosteroid and IFN-α treatment have no significant effect on tumor burden, but no more anaphylactic shock occurred.

Conclusions: Cladribine and imatinib are recommended to treat SM patients to obtain a better therapeutic effect. Maybe allogeneic hematopoietic stem cell transplantation is a cure for SM.