Intraoperative hemolysis. The initial manifestation of glucose-6-phosphate dehydrogenase deficiency.

Unexpected intraoperative hemolysis suggestive of a hemolytic transfusion reaction was the initial clinical manifestation of Mediterranean type glucose-6-phosphate dehydrogenase (G-6-PD) deficiency in a 50-year-old Indian man. There was no evidence for immune mediated hemolysis since no unexpected RBC antibodies were found, and results of the direct antiglobulin test remained negative. Analysis of preoperative blood specimens demonstrated complete absence of G-6-PD activity. No etiologic agent clearly associated with G-6-PD hemolysis could be identified. Testing for G-6-PD deficiency should be considered when unexpected hemolysis occurs intraoperatively.