Thrombotic thrombocytopenic purpura causing sudden, unexpected death--a series of eight patients

Eight patients were presented to the medical examiner after dying suddenly and unexpectedly from thrombotic thrombocytopenic purpura. Compared with patients who die after prolonged hospitalization and treatment, these patients showed fewer neurologic symptoms and correspondingly fewer or no microthrombi within the brain. Only four of eight subjects developed fever, which further contributed to misdiagnosis. The differences in clinical presentation between our cases and most published series is striking and may be explained by shorter duration and no treatment. Each case contained the characteristic histology of thrombotic thrombocytopenic purpura. Ischemic injury to the heart and conduction system was the most likely mechanism of sudden death. Included in this series is a patient with acquired immunodeficiency syndrome (AIDS) diagnosed at autopsy, a concurrence that is now appearing more frequently in the medical literature.