Intraoperative Death Due to Thrombotic Thrombocytopenic Purpura (TTP)-related Acute Myocardial Failure in a Patient Undergoing Laparoscopic Splenectomy

Abstract

Thrombotic thrombocytopenic purpura (TTP) is an uncommon disorder characterized by a pentad of microangiopathic hemolytic anemia, thrombocytopenia, renal dysfunction, fever, and a fluctuating neurologic syndrome. Splenectomy is performed for patients who are refractory to plasma therapy and for relapsing TTP. We describe a case of a patient who died due to intramyocardial hemorrhage after undergoing laparoscopic splenectomy for TTP resistant to treatment with plasmapheresis. A 52-year-old woman was admitted with ecchymoses, low platelet count, weakness of left face and upper extremity, and a presumptive diagnosis of TTP. Vital signs were stable. White blood count was 7800/microL, hemoglobin 7.9 g/dL, and platelet count of 13,000/microL. Her basic metabolic panel and liver function tests were normal. Further laboratory workup confirmed the diagnosis of TTP. The patient was initially treated with plasmapheresis and high dose steroid therapy but underwent an emergent laparoscopic splenectomy due to refractory TTP. At the end of the uneventful procedure, the patient suffered a cardiac arrest and died. Autopsy concluded that the death was from myocardial failure due to extensive myocardial hemorrhage secondary to TTP. There are several published case reports of sudden death due to cardiac involvement in TTP. However, intraoperative mortality is not reported. We conclude that TTP-related acute heart failure may represent an extremely important clinical risk in these patients who are undergoing surgery.