Recurrent small bowel ischemia in a patient with paroxysmal nocturnal hemoglobinuria

Background. A 33-year-old female presented with a history of recurrent epigastric pain and vomiting of bile, accompanied by duodenal wall thickening seen on an abdominal CT scan and a congestive, bluish and ischemic-looking duodenal mucosa as visualized by endoscopy. The patient's medical history was notable for paroxysmal nocturnal hemoglobinuria diagnosed 15 years before. The patient was usually treated with danazol and warfarin and she received regular blood transfusions for hemolytic anemia. Investigations. Medical history and physical examination. Blood tests, coagulation study, search for thrombophilic disorders and small vessel vasculitis. Stool culture and virology. Test for autoantibodies. Abdominal CT scan. Upper gastrointestinal endoscopy with duodenal biopsies. Diagnosis. Small bowel ischemia complicating paroxysmal nocturnal hemoglobinuria. Management. Treatment with warfarin was started but ischemic episodes recurred despite appropriate anticoagulation. Treatment with the complement inhibitor eculizumab was then proposed.