Intestinal ischemia complicating paroxysmal nocturnal hemoglobinuria.

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disorder of hematopoiesis characterized by pancytopenia, intravascular hemolysis, and bone marrow aplasia. It is manifested by repeated episodes of hemoglobinuria and a tendency for the development of widespread venous thrombosis, which may affect unusual sites, such as hepatic and mesenteric veins.1 Many patients suffer from recurrent attacks of abdominal pain that are assumed to be secondary to intestinal venous thrombosis. However, there is little direct evidence to support this hypothesis. We describe here a rare case of PNH: a patient who developed abdominal pain with clinical, endoscopic, and histologic findings suggestive of ischemic small bowel disease and who survived this severe complication. To our knowledge, this is the first report showing serial endoscopic images of this complication in a PNH patient.