Systemic mastocytosis presenting as intraoperative anaphylaxis with atypical features: a report of two cases

Two cases of perioperative cardiovascular collapse are presented that were associated with markedly elevated mast cell tryptase levels shortly after the event, leading to the assumption that an immunoglobin E-mediated, drug-induced anaphylaxis had occurred. However, the clinical picture in both cases was atypical and subsequent skin testing failed to identify a triggering drug. Further blood tests, some weeks later, revealed persistently elevated baseline levels of mast cell tryptase. In both cases bone marrow biopsy and genetic testing confirmed the diagnosis of mastocytosis. We present evidence and speculate that mast cell degranulation was triggered by tourniquet release in the first case and by exposure to peanuts in the second. An atypical presentation of anaphylaxis should alert the anaesthetist to the possibility of previously undiagnosed mastocytosis.