Difference between revisions of "Common variable immunodeficiency"
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*[[Plasma cells]] absent (or reduced) - '''key feature'''.<ref name=pmid20629103>{{cite journal |author=Agarwal S, Smereka P, Harpaz N, Cunningham-Rundles C, Mayer L |title=Characterization of immunologic defects in patients with common variable immunodeficiency (CVID) with intestinal disease |journal=Inflamm Bowel Dis |volume= |issue= |pages= |year=2010 |month=July |pmid=20629103 |doi=10.1002/ibd.21376 |url=}}</ref> | *[[Plasma cells]] absent (or reduced) - '''key feature'''.<ref name=pmid20629103>{{cite journal |author=Agarwal S, Smereka P, Harpaz N, Cunningham-Rundles C, Mayer L |title=Characterization of immunologic defects in patients with common variable immunodeficiency (CVID) with intestinal disease |journal=Inflamm Bowel Dis |volume= |issue= |pages= |year=2010 |month=July |pmid=20629103 |doi=10.1002/ibd.21376 |url=}}</ref> | ||
*+/-Lymphoid aggregates. | *+/-Lymphoid aggregates. | ||
*+/-Granulomatous disease ~8-22%.<ref name=pmid19716342>{{Cite journal | last1 = Ardeniz | first1 = O. | last2 = Cunningham-Rundles | first2 = C. | title = Granulomatous disease in common variable immunodeficiency. | journal = Clin Immunol | volume = 133 | issue = 2 | pages = 198-207 | month = Nov | year = 2009 | doi = 10.1016/j.clim.2009.05.001 | PMID = 19716342 }}</ref> | |||
DDx: | DDx: | ||
Revision as of 17:48, 18 March 2016
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.