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.


METHODS
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.


METHODS
Small bowel ischemia complicating paroxysmal nocturnal hemoglobinuria.


RESULTS
Treatment with warfarin was started but ischemic episodes recurred despite appropriate anticoagulation. Treatment with the complement inhibitor eculizumab was then proposed.
Torres
Joana
J
Clinique des Maladies de l'Appareil Digestif et de la Nutrition, Hôpital Claude Huriez, Rue Michel Polonovski, 59037 Lille Cedex, France.
De Vroey
Bénédicte
B
Noël
Marie-Pierre
MP
Notteghem
Bertrand
B
Colombel
Jean-Frédéric
JF
D000328:Adult; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D061067:Antibodies, Monoclonal, Humanized; D004378:Duodenal Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D004386:Duodenum / Q000098:blood supply*; D005260:Female; D006457:Hemoglobinuria, Paroxysmal / Q000150:complications*; D006801:Humans; D007413:Intestinal Mucosa / Q000473:pathology; D007511:Ischemia / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D012008:Recurrence; D016896:Treatment Outcome
D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; C481642:eculizumab