Paroxysmal nocturnal haemoglobinuria

Hunter, Judith R.; Nelson, Mark

doi:10.1007/bf02943686

Cited by 3 publications

(2 citation statements)

References 16 publications

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“…Chrobak reported one patient with intestinal infarction in 1963 who underwent two separate ileal resections and finally an ileostomy over a three month period prior to death [30]. In 1966, another patient underwent resection of infarcted duodenum and multiple areas of jejunum but survived only 8 days [32]. Two other PNH patients have been reported with possible intestinal ischemia or infarction as diagnosed by X-ray surviving (2 months, 2 years) with conservative management only [33,34].…”

Section: Discussionmentioning

confidence: 98%

Intestinal infarction caused by paroxysmal nocturnal hemoglobinuria

1984

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A patient with paroxysmal nocturnal hemoglobinuria of 14 years duration presented with severe abdominal pain and fever. On admission, his hematocrit had fallen to 19% from his usual level of approximately 30%, and stools were positive for occult blood. Dilated loops of small bowel with air fluid levels were noted on radiographs, and sonography revealed free intraperitoneal fluid. Several sections of gangrenous jejunum and ileum were resected at exploratory laparotomy, and mesenteric venous thromboses were present. This is the second report of pathologically proven intestinal infarction in a patient with paroxysmal nocturnal hemoglobinuria who has survived this abdominal emergency with surgical intervention. Although abdominal pain in patients with paroxysmal nocturnal hemoglobinuria has frequently been attributed to mesenteric venous thromboses, this has rarely been documented either during life or at autopsy.

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