Common variable immunodeficiency

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

General


Labs in a series of 8 patients:[3]

  • IgG 2.5 +/-0.6 g/L.
  • IgA 0.23 +/-0.1 g/L.
  • IgM 0.17 +/-0.1 g/L.

Gross

Duodenum:

  • Nodular mucosa - due to lymphoid hyperplasia.

Microscopic

Features - general:

  • Plasma cells absent (or reduced) - key feature.[2]
  • +/-Lymphoid aggregates.
  • +/-Granulomatous disease ~8-22%.[4]

DDx:

Features by site in the GI tract:[5]

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. 1.0 1.1 Gathmann, B.; Mahlaoui, N.; Gérard, L.; Oksenhendler, E.; Warnatz, K.; Schulze, I.; Kindle, G.; Kuijpers, TW. et al. (Jul 2014). "Clinical picture and treatment of 2212 patients with common variable immunodeficiency.". J Allergy Clin Immunol 134 (1): 116-26. doi:10.1016/j.jaci.2013.12.1077. PMID 24582312.
  2. 2.0 2.1 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.
  3. 3.0 3.1 Dong, J.; Liang, H.; Wen, D.; Wang, J. (Mar 2016). "Adult Common Variable Immunodeficiency.". Am J Med Sci 351 (3): 239-43. doi:10.1016/j.amjms.2015.12.010. PMID 26992251.
  4. 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.
  5. 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.