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.
Cartilage Hair Hypoplasia - Patients can develop autoimmune enteropathy and Hirschsprung disease. Other autoimmune manifestations include ITP, hemolytic anemia, and juvenile idiopathic arthritis.