A 52-year-old female with hypertension and type 2 diabetes mellitus presented to the hospital with severe abdominal pain, nausea, and vomiting for 24 hours. On physical examination she had fever and tenderness to palpation in her epigastrium and left upper quadrant. Imaging of her abdomen revealed an ileus. Lab work revealed anemia, severe thrombocytopenia, and acute renal failure; further evaluation showed hypoalbuminemia, hematuria, hyperbilirubinemia, elevated lactate dehydrogenase, and evidence of acute pancreatitis. She was admitted to the hospital for treatment of acute pancreatitis and was given intravenous fluids, antiemetics, and analgesics. Over the next 48 hours she had little improvement in her symptoms; the diagnosis of thrombotic thrombocytopenic purpura (TTP) was made in consultation with a hematologist, and the patient was transferred to a tertiary care hospital for plasmapheresis. Prior to initiation of therapy, however, the patient abruptly developed bradycardia with hypotension and expired. Autopsy findings included nonocclusive coronary artery disease, subepicardial petechiae, endocardial hemorrhage, and vascular microthrombi with associated myocardial necrosis. Sections of lung tissue revealed pulmonary congestion and edema. The characteristic pathological finding in patients with TTP is platelet-rich thrombus formation in the microvasculature of multiple organ systems. Commonly affected tissues include the adrenal glands, pancreas, kidneys, the central nervous system, and the heart. Cardiac involvement is common and was included in the original description of the disorder in 1924 by Moschcowitz. Clinical findings typical of myocardial ischemia are often absent, with a minority of patients reporting chest pain. Cardiac involvement as evidenced by serological myocardial injury biomarkers appears to be more common. Modern therapy with plasmapheresis, with or without the addition of glucocorticoids, has led to a significant reduction in mortality. Despite this improvement in clinical outcomes, histological examination of autopsy specimens shows that myocardial thrombotic involvement likely continues to occur in all patients, and in some cases myocardial infarction is a contributing factor to death. Although it is currently unclear how cardiac injury relates to prognosis in TTP, cardiac monitoring is reasonable given the occasional severity of its effects when myocardium is involved.
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