Histamine levels and cardiovascular responses during splenectomy and splenorenal shunt formation in a patient with systemic mastocytosis.

Systemic mastocytosis is a rare disorder characterised by the infiltration of many tissues by abnormal numbers of mast cells. Life-threatening episodes of bronchospasm and hypotension are common in response to a variety of triggers which cause mast cell degranulation. We present the case history of a patient with severe mast cell disease who successfully underwent splenectomy and portocaval anastomosis. Peri-operative therapy was directed towards mast cell stabilisation, and histamine-releasing drugs were avoided. Cardiovascular data, together with blood histamine levels and thrombin times, indicated that mast cell degranulation did not occur. A review of the current literature concerning mast cell disease as it relates to anaesthesia is presented and suggestions for the safe management of such cases are given.