intestinal perforation in a patient with paroxysmal nocturnal hemoglobinuria

Paroxysmal nocturnal hemoglobinuria (pnh) is a rare hematologic disorder that manifests with hemolytic anemia, thrombosis, and peripheral blood cytopenias.acute abdominal pain is one of the pnh clinical manifestations due to venous thrombosis of intra-abdominal sites including hepatic, portal, mesenteric, and splenic veins.eculizumaband allogeneic bone marrow transplantation (bmt) arethe only widely effective therapies for these patients. we report a case of pnh disorder which presented with abdominal pain and mild tenderness. abdominal x ray and sonography revealed intra-peritonealfree fluid and air. at laparotomy, an ischemic segment of jejunum with stricture and perforationwith mesenteric venous thrombosis were found. the patient also had a history of hepatic vein thrombosis two years before.