Gastrointestinal angiodysplasia in congenital platelet dysfunction.

ABSTRACT

We herein report three cases of repeated massive bleeding from the stomach and small bowel. One patient suffered from both thrombasthenia (type II) and von Willebrand disease (type 1) simultaneously. Two others had Bernard-Soulier's syndrome (BSS). One patient with BSS had bleeding from gastric angiodysplasia and was treated endoscopically by clipping. The other patients had massive bleeding from the small intestine, and had partial resection of the affected small intestine. Histologically, irregular dilatation and proliferation of the blood vessels were demonstrated in the submucosa in bleeding spots from a resected small intestine, and these findings were consistent with the features of acquired angiodysplasia. The development of gastrointestinal angiodysplasia may not only be associated with a dysfunction of von Willebrand factor but also with that of platelets.