CVID

 

 

 

 

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.  

CVID
Chronic Granulomatous Disease
Leukocyte Adhesion Deficiency
Chromosome 22q11.2 Deletion
CVID

 
 Hermansky Pudlak Syndrome

 

 

Hermansky-Pudlak syndrome - Patients with granulomatous enterocolitis mimicking crohns disease have been reported. Patients also have oculocutaneous hypopigmentation, neutropenia, and bleeding disorder. 

HERMANSKY PUDLAK SYNDROME