Common variable immunodeficiency
Common variable immunodeficiency, abbreviated CVID, is a group of genetic disorders characterized by deficient antibody production.
General
Features:[1]
- Recurrent sinus and pulmonary infections.
- Inflammatory bowel disease-like disease.
Gross
Duodenum:
- Nodular mucosa - due to lymphoid hyperplasia.
Microscopic
Features - general:
- Plasma cells absent (or reduced) - key feature.[1]
- +/-Lymphoid aggregates.
- +/-Granulomatous disease ~8-22%.[2]
DDx:
Features by site in the GI tract:[3]
| Site | Features |
|---|---|
| Esophagus | intraepithelial PMNs & candida +/-abundant intraepithelial lymphocytes |
| Stomach | lack of plasma cells (~2/3 of individuals), lymphoid aggregates (common), +/-lymphocytic gastritis, +/-intraepithelial neutrophils, +/-infections (CMV, H. pylori, cryptosporidium) |
| Small bowel | villous blunting (>80% of individuals), decreased plasma cells (~2/3 of individuals), lymphoid aggregates (~1/2 of individuals), +/-neutrophils + infections (CMV, cryptosporidium) |
| Large bowel | decreased plasma cells, lymphoid aggregates, +/-apparent apoptosis |
See also
References
- ↑ 1.0 1.1 Agarwal S, Smereka P, Harpaz N, Cunningham-Rundles C, Mayer L (July 2010). "Characterization of immunologic defects in patients with common variable immunodeficiency (CVID) with intestinal disease". Inflamm Bowel Dis. doi:10.1002/ibd.21376. PMID 20629103.
- ↑ Ardeniz, O.; Cunningham-Rundles, C. (Nov 2009). "Granulomatous disease in common variable immunodeficiency.". Clin Immunol 133 (2): 198-207. doi:10.1016/j.clim.2009.05.001. PMID 19716342.
- ↑ Daniels, JA.; Lederman, HM.; Maitra, A.; Montgomery, EA. (Dec 2007). "Gastrointestinal tract pathology in patients with common variable immunodeficiency (CVID): a clinicopathologic study and review.". Am J Surg Pathol 31 (12): 1800-12. doi:10.1097/PAS.0b013e3180cab60c. PMID 18043034.