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Recurrent small bowel ischemia in a patient with paroxysmal nocturnal hemoglobinuria

Nature Reviews Gastroenterology & Hepatology volume 7, pages 410–414 (2010)Cite this article

Subjects

Abstract

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.

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Figure 1: Abdominal CT scan of the case patient at admission.
Figure 2: Endoscopic images of duodenum at admission.
Figure 3: Endoscopic image of the duodenum taken during a recurrent episode of abdominal pain, nausea and vomiting.

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Acknowledgements

Written consent for publication was obtained from the patient.

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Authors and Affiliations

  1. Clinique des Maladies de l'Appareil Digestif et de la Nutrition, Hôpital Claude Huriez, Rue Michel Polonovski, 59037 Lille Cedex, France

    Joana Torres, Bénédicte De Vroey & Jean-Frédéric Colombel

  2. Clinique des Maladies du Sang, Hôpital Claude Huriez, Rue Michel Polonovski, 59037 Lille Cedex, France

    Marie-Pierre Noël

  3. Résidence du Théâtre, 16 Rue du Coq Français, Roubaix, 59100, France

    Bertrand Notteghem

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  1. Joana Torres
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  5. Jean-Frédéric Colombel
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Correspondence to Jean-Frédéric Colombel.

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Torres, J., De Vroey, B., Noël, MP. et al. Recurrent small bowel ischemia in a patient with paroxysmal nocturnal hemoglobinuria. Nat Rev Gastroenterol Hepatol 7, 410–414 (2010). https://doi.org/10.1038/nrgastro.2010.59

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