[Paroxysmal nocturnal hemoglobinuria associated with intestinal ischemia leading to small bowel perforation].
Paroxysmal nocturnal hemoglobinuria is rarely associated with intestinal complications. We report a case of paroxysmal nocturnal hemoglobinuria with small bowel ischemia leading to ileal perforation. In the literature, an ulcerative jejuno-ileitis has been reported in 6 cases of intestinal ischemia, due to thrombosis of mucosal small vessels. This disease is usually revealed by abdominal pain. Based on published cases and our observation of intestinal ischemia leading to small bowel perforation, surgery should be considered as the first-line treatment, especially when small bowel lesions are limited.
Quentin
Vincent
V
Service des Maladies de l'Appareil Digestif, CHU Pontchaillou, 35033 Rennes.
Dinasquet
Michel
M
Rioux-Leclercq
Nathalie
N
de Lajarte-Thirouard
Anne-Sophie
AS
Lotrian
Dominique
D
Lamy
Thierry
T
Bernard
Marc
M
Olivié
Damien
D
Gosselin
Michel
M
Bretagne
Jean-François
JF
Heresbach
Denis
D
D000328:Adult; D005260:Female; D006457:Hemoglobinuria, Paroxysmal / Q000150:complications*; D006801:Humans; D007077:Ileal Diseases / Q000209:etiology*; D007416:Intestinal Perforation / Q000209:etiology*; D007421:Intestine, Small / Q000098:blood supply*; D007511:Ischemia / Q000150:complications*