Gastrointestinal Ischemia as the Initial Presentation of Thrombotic Thrombocytopenic Purpura

Abstract

The hallmark of thrombotic thrombocytopenic purpura (TTP) is systemic platelet thrombi in the microcirculation with ensuing end-organ damage. While gastrointestinal manifestations are not typical of TTP, our data suggest that abdominal complaints may be more common than previously recognized (Blood. 2010;116:612). We describe 2 patients with fatal TTP (ADAMTS-13 activity level <5% due to inhibitor) and intestinal ischemia. The first was a 54-year-old white woman admitted to another hospital with nausea, vomiting, abdominal pain, diarrhea, hemolytic anemia, and thrombocytopenia. After receiving platelets and packed RBCs, she was transferred to our institution for plasma exchange. She died following the first procedure, 3 days after presentation. Postmortem examination revealed microthrombi with extensive hemorrhage and necrosis throughout her gastrointestinal tract. The second patient was a 58-year-old black man with abdominal pain and imaging suggestive of small bowel obstruction. With an initial platelet count of 211,000/μL, he underwent resection of a segment of ischemic small bowel. The next day, he developed thrombocytopenia followed by altered mental status and subsequent repeated episodes of cardiac arrest with anoxic brain injury. Despite plasma exchange, he died on hospital day 10. Autopsy showed multiple subepicardial infarctions with microthrombi within intramural coronary arteries. Microthrombi were also present in the kidneys, bowel, and brain. Additionally, review of microscopic sections of the surgically resected bowel revealed platelet and fibrin thrombi in the microvasculature, confirming TTP was present at the time of surgery. TTP is a difficult diagnosis as it can cause nonspecific signs and symptoms. Without plasma exchange, TTP carries a 90% risk of death. These 2 cases illustrate that gastrointestinal complaints can be the only manifestations of this rare disease. Pathologists reviewing ischemic bowel resections must be aware of TTP and report platelet microthrombi to their clinical colleagues. These histologic findings should alert them of the possibility of TTP.