Recurrent bowel infarction in paroxysmal nocturnal haemoglobinuria

Abstract

A man of 43 was referred after 24 hours of feeling generally unwell with intermittent rigors. PNH had been diagnosed 18 years previously, and regular blood transfusions for his anaemia had resulted in iron-overload diabetes mellitus. 8 years before the present episode he had had a small-bowel resection for ischaemia secondary to venous thrombosis. On examination his abdomen was distended and slightly tender in the left lower quadrant, without obvious peritonism. Haemoglobin was 6.4 g/dL and platelet count 166109/L. Blood indices were otherwise normal. A plain abdominal X-ray showed dilated small-bowel loops. Adhesional small-bowel obstruction was diagnosed and initial management was conservative. After three days the abdominal distention had not resolved and a CT scan (Figure 1) showed gross small-bowel dilatation with an area of collapsed small bowel distally, highly suggestive of a mechanical obstruction. At laparotomy, with bloodtransfusion and platelet cover, he was found to have a segment of gangrenous small bowel with a stricture. This was resected and the bowel was anastomosed by an extramucosal technique. The patient spent the first three postoperative days on the intensive care unit for close monitoring, and received total parenteral nutrition for five days until enteral feeding was started. No further blood or platelet transfusions were required. Histopathological examination of the operative specimen showed extensive haemorrhagic infarction secondary to venous thrombosis.