Difference between revisions of "Common variable immunodeficiency"
Jump to navigation
Jump to search
(→Microscopic: make table) |
|||
| Line 20: | Line 20: | ||
*[[Graft versus host disease]]. | *[[Graft versus host disease]]. | ||
Features by site in the GI tract:<ref name=pmid18043034>{{Cite journal | last1 = Daniels | first1 = JA. | last2 = Lederman | first2 = HM. | last3 = Maitra | first3 = A. | last4 = Montgomery | first4 = EA. | title = Gastrointestinal tract pathology in patients with common variable immunodeficiency (CVID): a clinicopathologic study and review. | journal = Am J Surg Pathol | volume = 31 | issue = 12 | pages = 1800-12 | month = Dec | year = 2007 | doi = 10.1097/PAS.0b013e3180cab60c | PMID = 18043034 }}</ref> | |||
Features | {| class="wikitable sortable" | ||
!Site | |||
!Features | |||
|- | |||
| Esophagus | |||
| intraepithelial PMNs & candida +/-abundant intraepithelial lymphocytes | |||
|- | |||
| Stomach | |||
| lack of [[plasma cell]]s (~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== | ==See also== | ||
Revision as of 04:50, 7 March 2013
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.
DDx:
Features by site in the GI tract:[2]
| 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.
- ↑ 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.