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Long-Term Evaluation of a Historical Cohort of Iranian Common Variable Immunodeficiency Patients Publisher Pubmed



Aghamohammadi A1 ; Abolhassani H1, 2 ; Latif A1 ; Tabassomi F1 ; Shokuhfar T1 ; Torabi Sagvand B1 ; Shahinpour S1 ; Mirminachi B1 ; Parvaneh N1 ; Movahedi M3 ; Gharagozlou M3 ; Sherkat R4 ; Amin R5 ; Aleyasin S5 Show All Authors
Authors
  1. Aghamohammadi A1
  2. Abolhassani H1, 2
  3. Latif A1
  4. Tabassomi F1
  5. Shokuhfar T1
  6. Torabi Sagvand B1
  7. Shahinpour S1
  8. Mirminachi B1
  9. Parvaneh N1
  10. Movahedi M3
  11. Gharagozlou M3
  12. Sherkat R4
  13. Amin R5
  14. Aleyasin S5
  15. Faridhosseini R6
  16. Jabbariazad F7
  17. Cheraghi T8
  18. Eslamian MH1, 3
  19. Khalili A1, 3
  20. Kalantari N9
  21. Shafiei A10
  22. Dabbaghzade A1, 3
  23. Khayatzadeh A1, 3
  24. Ebrahimi M1, 3
  25. Razavinejad D1
  26. Bazregari S1, 3
  27. Ebrahimi M1, 3
  28. Ghaffari J11
  29. Bemanian MH12
  30. Behniafard N1, 10
  31. Kashef S13
  32. Mohammadzadeh I14
  33. Hammarstrom L2
  34. Rezaei N1

Source: Expert Review of Clinical Immunology Published:2014


Abstract

Objectives: Common variable immune deficiency (CVID) is the most frequent form of symptomatic primary immunodeficiency disease, characterized by hypogammaglobulinemia, recurrent infections and increased predisposition to autoimmunity and malignancies. The aim of this study was to reconsider important points of previously performed studies on Iranian CVID patients diagnosed and followed from 1984 to 2013. Methods: Diagnosis was made using approved criteria including reductions of serum levels of immunoglobulins and exclusion of well-known single gene defects in individuals with an age >4 years and evidence of specific antibody deficiency. Results: Detailed information on demographic data, survival rates, clinical phenotypes, immunologic and genetic data and treatment of 173 patients are provided. The early onset presentation (74.5%) and rate of consanguineous marriage (61.2%) were considerably higher in our cohort. Our study revealed clinically related correlations regarding consanguinity, the population of naive CD4+T cells and switched-memory B cells, cytokine levels and special genetic factors (including HLA and AID genes). Conclusion: Despite current efforts, more comprehensive studies are needed, especially for classification and investigation of the genetic background and prognostic factors for patients with CVID in order to better managment and followup of patinets. © 2014 Informa UK, Ltd.
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