Common variable immunodeficiency

From Libre Pathology
Jump to navigation Jump to search
The printable version is no longer supported and may have rendering errors. Please update your browser bookmarks and please use the default browser print function instead.

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.