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.