Yıl: 2020 Cilt: 18 Sayı: 1 Sayfa Aralığı: 30 - 37 Metin Dili: İngilizce DOI: 10.21911/aai.504 İndeks Tarihi: 06-11-2020

Five Years of Experience in a Single Center: Retrospective Analysis of Adult Patients with Common Variable Immunodeficiency

Öz:
Objective: Common Variable Immune Deficiency (CVID) is a heterogeneous immune disorder characterized by impaired and/or inadequate B cell differentiation with hypogammaglobulinemia. It is characterized by frequent and recurrent respiratory infections, autoimmune disorders, chronic lung diseases, granulomatous diseases, and increased risk for lymphoid malignancies.Materials and Methods: The medical records of 47 patients (22 females, 25 males) who had been followed up at our clinic and had sufficient data in their files were retrospectively reviewed. Patients were diagnosed with CVID according to the ESID (European Society for Immunodeficiency) criteria.Results: The median age of the patients was 32 (19-65) years. The most frequent clinical presentation of the patients was with recurrent upper respiratory infections (46%), pneumonia (29.8%), otitis media (23.4%) and chronic sinusitis (17%). During the follow-up period, 17 patients (36.8%) developed autoimmune complications, 14 (29.8%) of whom had autoimmune cytopenia. A total of 26 patients (55.3%) had bronchiectasis confirmed with computed tomography of the thorax. Lymphopenia was detected in 13 patients (27.7%). The median immunoglobulin level at the time of diagnosis was IgG 2.77 (0.33-6.90) g/L, IgM 0.31 (0.06-5.99) g/L, and IgA 0.25 (0.01-5.02) g/L.Conclusion: CVID is very heterogeneous in terms of both clinical and laboratory features. Moreover, it is more common than expected, particularly in adulthood. The centers dealing with CVID should share their experiences in order to increase awareness among physicians.
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  • 1. Cunningham-Rundles C. Clinical and immunologic analyses of 103 patients with common variable immunodeficiency. J Clin Immunol 1989;9(1):22-33.
  • 2. Cunningham-Rundles C, Bodian C. Common variable immunodeficiency: Clinical and immunological features of 248 patients. Clin Immunol 1999;92(1):34-48.
  • 3. Hermaszewski RA, Webster AD. Primary hypogammaglobu-linaemia: A survey of clinical manifestations and complications. Q J Med 1993;86(1):31-42.
  • 4. Sneller MC, Strober W, Eisenstein E, Jaffe JS, Cunningham-Rundles C. NIH conference. New insights into common variable immunodeficiency. Ann Intern Med 1993;118(9):720-30.
  • 5. Spickett GP, Farrant J, North ME, Zhang JG, Morgan L, Webster AD. Common variable immunodeficiency: How many diseases? Immunol Today 1997;18(7):325-8.
  • 6. Janeway CA, Apt L, Gitlin D. Agammaglobulinemia. Trans Assoc Am Physicians 1953;66:200-2.
  • 7. Oksenhendler E, Gerard L, Fieschi C, Malphettes M, Mouillot G, Jaussaud R, et al. Infections in 252 patients with common variable immunodeficiency. Clin Infect Dis 2008;46(10):1547-54.
  • 8. Urschel S, Kayikci L, Wintergerst U, Notheis G, Jansson A, Belohradsky BH. Common variable immunodeficiency disorders in children: Delayed diagnosis despite typical clinical presentation. J Pediatr 2009;154(6):888-94.
  • 9. Caliskaner AZ, Reisli I, Arslan S, Ucar R, Ataseven H, Selcuk NY. Common variable immunodeficiency in adults requires reserved protocols for long-term follow-up. Turk J Med Sci 2016;46(2):430-6.
  • 10. Muşabak UH, Demirel F, Yesillik S, Baysan A, Selcuk A, Kartal O, et al. Adults with common variable immunodeficiency: A single-center experience. Turk J Med Sci 2017;47(1):1-12.
  • 11. Conley ME, Notarangelo LD, Etzioni A. Diagnostic criteria for primary immunodeficiencies. Representing PAGID (Pan-American Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies). Clin Immunol 1999;93(3):190-7.
  • 12. Hollowell JG, van Assendelft OW, Gunter EW, Lewis BG, Najjar M, Pfeiffer C; Centers for Disease Control and Prevention, National Center for Health Statistics. Hematological and iron-related analytes--reference data for persons aged 1 year and over: United States, 1988-94. Vital Health Stat 11 2005;(247):1-156.
  • 13. Valent P. Low blood counts: Immune mediated, idiopathic, or myelodysplasia. Hematology Am Soc Hematol Educ Program 2012;2012:485-91.
  • 14. Ameratunga R, Woon ST, Gillis D, Koopmans W, Steele R. New diagnostic criteria for common variable immune deficiency (CVID), which may assist with decisions to treat with intravenous or subcutaneous immunoglobulin. Clin Exp Immunol 2013;174(2):203-11.
  • 15. Martins TB, Bandhauer ME, Bunker AM, Roberts WL, Hill HR. New childhood and adult reference intervals for total IgE. J Allergy Clin Immunol 2014;133(2):589-91.
  • 16. Azar AE, Ballas ZK. Evaluation of the adult with suspected immunodeficiency. Am J Med 2007;120(9):764-8.
  • 17. Orange JS, Ballow M, Stiehm ER, Ballas ZK, Chinen J, De La Morena M, et al. Use and interpretation of diagnostic vaccination in primary immunodeficiency: a working group report of the Basic and Clinical Immunology Interest Section of the American Academy of Allergy, Asthma & Immunology. J Allergy Clin Immunol 2012;130(3 Suppl):S1-24.
  • 18. Rudolf-Oliveira RC, Goncalves KT, Martignago ML, Mengatto V, Gaspar PC, de Moraes AC, et al. Determination of lymphocyte subset reference ranges in peripheral blood of healthy adults by a dual-platform flow cytometry method. Immunol Lett 2015;163(1):96-101.
  • 19. Gathmann B, Mahlaoui N, Ceredih, Gerard L, Oksenhendler E, Warnatz K, et al. Clinical picture and treatment of 2212 patients with common variable immunodeficiency. J Allergy Clin Immunol 2014;134(1):116-26.
  • 20. Ardeniz O, Basoglu OK, Gunsar F, Unsel M, Bayraktaroglu S, Mete N, et al. Clinical and immunological analysis of 23 adult patients with common variable immunodeficiency. J Investig Allergol Clin Immunol 2010;20(3):222-36.
  • 21. Thickett KM, Kumararatne DS, Banerjee AK, Dudley R, Stableforth DE. Common variable immune deficiency: Respiratory manifestations, pulmonary function and high-resolution CT scan findings. QJM 2002;95(10):655-62.
  • 22. Hince M, Sakkal S, Vlahos K, Dudakov J, Boyd R, Chidgey A. The role of sex steroids and gonadectomy in the control of thymic involution. Cell Immunol 2008;252(1-2):122-38.
  • 23. Pido-Lopez J, Imami N, Aspinall R. Both age and gender affect thymic output: More recent thymic migrants in females than males as they age. Clin Exp Immunol 2001;125(3):409-13.
  • 24. Chapel H, Lucas M, Lee M, Bjorkander J, Webster D, Grimbacher B, et al. Common variable immunodeficiency disorders: Division into distinct clinical phenotypes. Blood. 2008;112(2):277-86.
  • 25. Quinti I, Soresina A, Spadaro G, Martino S, Donnanno S, Agostini C, et al. Long-term follow-up and outcome of a large cohort of patients with common variable immunodeficiency. J Clin Immunol 2007;27(3):308-16.
  • 26. Boileau J, Mouillot G, Gerard L, Carmagnat M, Rabian C, Oksenhendler E, et al. Autoimmunity in common variable immunodeficiency: Correlation with lymphocyte phenotype in the French DEFI study. J Autoimmun 2011;36(1):25-32.
  • 27. Wang J, Cunningham-Rundles C. Treatment and outcome of autoimmune hematologic disease in common variable immunodeficiency (CVID). J Autoimmun 2005;25(1):57-62.
  • 28. Cunningham-Rundles C, Siegal FP, Cunningham-Rundles S, Lieberman P. Incidence of cancer in 98 patients with common varied immunodeficiency. J Clin Immunol 1987;7(4):294-9.
  • 29. Aghamohammadi A, Allahverdi A, Abolhassani H, Moazzami K, Alizadeh H, Gharagozlou M, et al. Comparison of pulmonary diseases in common variable immunodeficiency and X-linked agammaglobulinaemia. Respirology 2010;15(2):289-95.
  • 30. Karaca NE, Severcan EU, Bilgin BG, Azarsiz E, Akarcan S, Gunaydin NC, et al. Familial inheritance and screening of first-degree relatives in common variable immunodeficiency and immunoglobulin A deficiency patients. Int J Immunopathol Pharmacol 2018;32:1-6.
  • 31. Cunningham-Rundles C. Common variable immune deficiency: Dissection of the variable. Immunol Rev 2019;287(1):145-61.
  • 32. Wehr C, Kivioja T, Schmitt C, Ferry B, Witte T, Eren E, et al. The EUROclass trial: Defining subgroups in common variable immunodeficiency. Blood 2008;111(1):77-85.
  • 33. Piqueras B, Lavenu-Bombled C, Galicier L, Bergeron-van der Cruyssen F, Mouthon L, Chevret S, et al. Common variable immunodeficiency patient classification based on impaired B cell memory differentiation correlates with clinical aspects. J Clin Immunol 2003;23(5):385-400.
  • 34. Giovannetti A, Pierdominici M, Mazzetta F, Marziali M, Renzi C, Mileo AM, et al. Unravelling the complexity of T cell abnormalities in common variable immunodeficiency.J Immunol 2007;178(6):3932-43.
  • 35. Oraei M, Aghamohammadi A, Rezaei N, Bidad K, Gheflati Z, Amirkhani A, et al. Naive CD4+ T cells and recent thymic emigrants in common variable immunodeficiency. J Investig Allergol Clin Immunol 2012;22(3):160-7.
  • 36. Bateman EA, Ayers L, Sadler R, Lucas M, Roberts C, Woods A, et al. T cell phenotypes in patients with common variable immunodeficiency disorders: Associations with clinical phenotypes in comparison with other groups with recurrent infections. Clin Exp Immunol 2012;170(2):202-11.
APA Aytekin G, YILDIZ E, ÇÖLKESEN F, Arslan S, CALISKANER A (2020). Five Years of Experience in a Single Center: Retrospective Analysis of Adult Patients with Common Variable Immunodeficiency. , 30 - 37. 10.21911/aai.504
Chicago Aytekin Gökhan,YILDIZ ERAY,ÇÖLKESEN FATİH,Arslan Sevket,CALISKANER Ahmet Zafer Five Years of Experience in a Single Center: Retrospective Analysis of Adult Patients with Common Variable Immunodeficiency. (2020): 30 - 37. 10.21911/aai.504
MLA Aytekin Gökhan,YILDIZ ERAY,ÇÖLKESEN FATİH,Arslan Sevket,CALISKANER Ahmet Zafer Five Years of Experience in a Single Center: Retrospective Analysis of Adult Patients with Common Variable Immunodeficiency. , 2020, ss.30 - 37. 10.21911/aai.504
AMA Aytekin G,YILDIZ E,ÇÖLKESEN F,Arslan S,CALISKANER A Five Years of Experience in a Single Center: Retrospective Analysis of Adult Patients with Common Variable Immunodeficiency. . 2020; 30 - 37. 10.21911/aai.504
Vancouver Aytekin G,YILDIZ E,ÇÖLKESEN F,Arslan S,CALISKANER A Five Years of Experience in a Single Center: Retrospective Analysis of Adult Patients with Common Variable Immunodeficiency. . 2020; 30 - 37. 10.21911/aai.504
IEEE Aytekin G,YILDIZ E,ÇÖLKESEN F,Arslan S,CALISKANER A "Five Years of Experience in a Single Center: Retrospective Analysis of Adult Patients with Common Variable Immunodeficiency." , ss.30 - 37, 2020. 10.21911/aai.504
ISNAD Aytekin, Gökhan vd. "Five Years of Experience in a Single Center: Retrospective Analysis of Adult Patients with Common Variable Immunodeficiency". (2020), 30-37. https://doi.org/10.21911/aai.504
APA Aytekin G, YILDIZ E, ÇÖLKESEN F, Arslan S, CALISKANER A (2020). Five Years of Experience in a Single Center: Retrospective Analysis of Adult Patients with Common Variable Immunodeficiency. Astım Allerji İmmünoloji, 18(1), 30 - 37. 10.21911/aai.504
Chicago Aytekin Gökhan,YILDIZ ERAY,ÇÖLKESEN FATİH,Arslan Sevket,CALISKANER Ahmet Zafer Five Years of Experience in a Single Center: Retrospective Analysis of Adult Patients with Common Variable Immunodeficiency. Astım Allerji İmmünoloji 18, no.1 (2020): 30 - 37. 10.21911/aai.504
MLA Aytekin Gökhan,YILDIZ ERAY,ÇÖLKESEN FATİH,Arslan Sevket,CALISKANER Ahmet Zafer Five Years of Experience in a Single Center: Retrospective Analysis of Adult Patients with Common Variable Immunodeficiency. Astım Allerji İmmünoloji, vol.18, no.1, 2020, ss.30 - 37. 10.21911/aai.504
AMA Aytekin G,YILDIZ E,ÇÖLKESEN F,Arslan S,CALISKANER A Five Years of Experience in a Single Center: Retrospective Analysis of Adult Patients with Common Variable Immunodeficiency. Astım Allerji İmmünoloji. 2020; 18(1): 30 - 37. 10.21911/aai.504
Vancouver Aytekin G,YILDIZ E,ÇÖLKESEN F,Arslan S,CALISKANER A Five Years of Experience in a Single Center: Retrospective Analysis of Adult Patients with Common Variable Immunodeficiency. Astım Allerji İmmünoloji. 2020; 18(1): 30 - 37. 10.21911/aai.504
IEEE Aytekin G,YILDIZ E,ÇÖLKESEN F,Arslan S,CALISKANER A "Five Years of Experience in a Single Center: Retrospective Analysis of Adult Patients with Common Variable Immunodeficiency." Astım Allerji İmmünoloji, 18, ss.30 - 37, 2020. 10.21911/aai.504
ISNAD Aytekin, Gökhan vd. "Five Years of Experience in a Single Center: Retrospective Analysis of Adult Patients with Common Variable Immunodeficiency". Astım Allerji İmmünoloji 18/1 (2020), 30-37. https://doi.org/10.21911/aai.504