CVID - Patients are at risk for developing a number of GI tract pathologies including lymphoid nodular hyperplasia and inflammatory bowel disease. An enteropathy with villous blunting (resembling celiac disease) may be present but does not respond to dietary gluten removal. The risk of developing GI tract malignancies (lymphoma, adenocarcinoma) is increased 50 fold.y.
Hermansky-Pudlak syndrome - Patients with granulomatous enterocolitis mimicking crohns disease have been reported. Patients also have oculocutaneous hypopigmentation, neutropenia, and bleeding disorder.