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.  

 XIAP Deficiency​


XIAP Deficiency patients can develop chronic hemorrhagic colitis.  This clinical feature has not been observed in patients with SAP Deficiency.