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.
DDx:
*[[Inflammatory bowel disease]].
*[[Celiac disease]].
*[[Graft versus host disease]].


===Esophagus===
===Esophagus===

Revision as of 04:43, 7 March 2013

Common variable immunodeficiency, abbreviated CVID, is a group of genetic disorders characterized by deficient antibody production.

General

Features:[1]

Gross

Duodenum:

  • Nodular mucosa - due to lymphoid hyperplasia.

Microscopic

Features - general:

DDx:

Esophagus

Features - esophagus:[2]

  • Intraepithelial PMNs & candida.
  • +/-Abundant intraepithelial lymphocytes.

Stomach

Features - stomach:[2]

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. 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. 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.