Difference between revisions of "Common variable immunodeficiency"
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*Recurrent sinus and pulmonary infections. | *Recurrent sinus and pulmonary infections. | ||
*[[Inflammatory bowel disease]]-like disease. | *[[Inflammatory bowel disease]]-like disease. | ||
==Gross== | |||
Duodenum: | |||
*Nodular mucosa - due to lymphoid hyperplasia. | |||
==Microscopic== | ==Microscopic== | ||
Features: | Features - general: | ||
*[[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. | |||
===Esophagus=== | |||
Features - esophagus:<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> | |||
*Intraepithelial PMNs & candida. | |||
*+/-Abundant intraepithelial lymphocytes. | |||
===Stomach=== | |||
Features - stomach:<ref name=pmid18043034/> | |||
*Lack of [[plasma cell]]s ~ 2/3 of individuals - '''key feature''' | |||
*Lymphoid aggregates - common. | |||
*+/-[[Lymphocytic gastritis]]. | |||
*+/-Intraepithelial neutrophils. | |||
*+/-Infections ([[CMV]], [[H. pylori]], [[cryptosporidium]]). | |||
===Small bowel=== | |||
Features - small bowel:<ref name=pmid18043034/> | |||
*Villous blunting > 80% of individuals. | |||
*Decreased plasma cells ~ 2/3 of individuals - '''key feature'''. | |||
*Lymphoid aggregates ~ 1/2 of individuals. | |||
*+/-Neutrophils + infections (CMV, cryptosporidium). | |||
===Large bowel=== | |||
Features:<ref name=pmid18043034/> | |||
*Decreased plasma cells - '''key feature'''. | |||
*Lymphoid aggregates. | |||
*+/-Apparent apoptosis. | |||
==See also== | ==See also== | ||
| Line 14: | Line 45: | ||
==References== | ==References== | ||
{{Reflist| | {{Reflist|2}} | ||
[[Category:Haematopathology]] | [[Category:Haematopathology]] | ||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
Revision as of 04:41, 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.
Esophagus
Features - esophagus:[2]
- Intraepithelial PMNs & candida.
- +/-Abundant intraepithelial lymphocytes.
Stomach
Features - stomach:[2]
- Lack of plasma cells ~ 2/3 of individuals - key feature
- Lymphoid aggregates - common.
- +/-Lymphocytic gastritis.
- +/-Intraepithelial neutrophils.
- +/-Infections (CMV, H. pylori, cryptosporidium).
Small bowel
Features - small bowel:[2]
- Villous blunting > 80% of individuals.
- Decreased plasma cells ~ 2/3 of individuals - key feature.
- Lymphoid aggregates ~ 1/2 of individuals.
- +/-Neutrophils + infections (CMV, cryptosporidium).
Large bowel
Features:[2]
- Decreased plasma cells - key feature.
- 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.
- ↑ 2.0 2.1 2.2 2.3 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.