Common Variable Immunodeficiency – GI Involvement
Definition
- Gastrointestinal changes associated with common variable immunodeficiency
Alternate/Historical Names
- Acquired hypogammaglobulinemia
- Adult-onset hypogammaglobulinemia
- Dysgammaglobulinemia
Diagnostic Criteria
- Markedly decreased plasma cells in 67% of cases
- Prominent lymphoid aggregates in 50-80% of cases
- Most common in large intestine
- Predominantly B cells in aggregates
- Single cell necrosis and increased apoptosis in 10-50% of cases
- Most prominent in large intestine
- Increased intraepithelial lymphocytes in 20-60% of cases
- Most prominent in stomach and small intestine
- Predominantly T cells
- Variable degrees of villous blunting in most small intestinal specimens that have increased intraepithelial lymphocytes
- Variable crypt distortion in 47% of colon specimens
- Non-caseating granulomas in 20% of large intestine cases
- Seen rarely in other sites
- Features of collagenous colitis seen in 12% of large intestine cases
- Intraepithelial neutrophils predominantly associated with infectious organisms
- Frequent GI infections
- Stomach and intestines
- Giardia
- Salmonella
- Campylobacter
- Cryptosporidium
- Cytomegalovirus
- Esophagus
- Candida
Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342
Original posting : November 12, 2009
