Clinical features and immunoglobulin replacement therapy outcomes of adults with common variable immunodeficiency: a single centre experience

Clinical features and immunoglobulin replacement therapy outcomes of adults with common variable immunodeficiency: a single centre experience

Background/aim: Common variable immunodeficiency (CVID) characterized by defective immunoglobulin production is the most prevalent form of symptomatic primary immunodeficiency (PID) in adults. We aimed to reveal the clinical features of adults with CVID and to evaluate the effects of immunoglobulin replacement treatment (IRT) on hemato-immunological findings. Materials and methods: This study included 26 adult patients receiving IRT. Two measurements of complete blood counts and major immunoglobulin levels obtained at the beginning-end of follow up period were used for comparisons. Lymphocyte subsets and B-cell subgroups were measured only at the time of presentation. Results: The most common complications were related to respiratory and digestive systems and organomegaly. Chronic diarrhoea and low body weight were positively correlated with the percentage of CD8+ T cells (p = 0.019 and p = 0.003, respectively) but negatively correlated with the CD4/CD8 ratio and the percentage of CD19+ B cells (p = 0.019 and p = 0.005 for both parameters, respectively). At the end of period, the distribution of haematological parameters significantly improved, and immunoglobulin M (IgM) level increased to detectable levels (p = 0.035). Conclusions: There are apparent relationships among chronic diarrhoea and low body weight, and deterioration of T and B cell immunity in adults with CVID. IRT improves the whole blood parameters and stimulates immunoglobulin M (IgM) production. The later effect supports the immunomodulatory feature of this therapy.Key words: Common variable immunodeficiency, intravenous immunoglobulins, diarrhoea

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  • 1. Abbott JK, Gelfand EW. Common variable immunodeficiency: diagnosis, management, and treatment. Immunology and Allergy Clinics of North America 2015; 35 (4): 637-658. doi: 10.1016/j.iac.2015.07.009.
  • 2. Conley ME, Notarangelo LD, Etzioni A. Diagnostic criteria for primary immunodeficiencies. Representing PAGID (PanAmerican Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies). Clinical ımmunology 1999; 93 (3): 190-197. doi: 10.1006/clim.1999.4799.
  • 3. Hooper AJ. The history and evolution of immunoglobulin products and their clinical indications. The Journal of Inherited Immune Disorders (LmyphoSign Journal) 2015; 2 (4): 181- 194. doi.org/10.14785/lpsn-2014-0025.
  • 4. Kazatchkine MD, Kaveri SV. Immunomodulation of autoimmune and inflammatory diseases with intravenous immunoglobulin. The New England Journal of Medicine 2001; 345 (10): 747-755. doi: 10.1056/NEJMra993360.
  • 5. Chaigne B, Mouthon L. Mechanisms of action of intravenous immunoglobulin. Transfusion and Apheresis Science 2016; 56 (1): 45-49. doi: 10.1016/j.transci.2016.12.017.
  • 6. Perez EE, Orange JS, Bonilla F, Chinen J, Chinn IK et al. Update on the use of immunoglobulin in human disease: A review of evidence. The Journal of Allergy and Clinical Immunology 2017; 139 (3S): 1-46. doi: 10.1016/j.jaci.2016.09.023.
  • 7. Maple-Brown LJ, Lawton PD, Hughes JT, Sharma SK, Jones GR et al. Study protocol-accurate assessment of kidney function in Indigenous Australians: aims and methods of the eGFR study. BMC Public Health 2010; 10: 80. doi: 10.1186/1471-2458-10- 80.
  • 8. Cunningham-Rundles C. How I treat common variable immune deficiency. Blood 2010; 116 (1): 7-15. doi: 10.1182/blood2010-01-254417.
  • 9. Oksenhendler E, Gerard L, Fieschi C, Malphettes M, Mouillot G et al. Infections in 252 patients with common variable immunodeficiency. Clinical Infectious Diseases 2008; 46 (10): 1547-1554. doi: 10.1086/587669.
  • 10. Malphettes M, Gérard L, Carmagnat M, Mouillot G, Vince N et al. Late-onset combined immune deficiency: a subset of common variable immunodeficiency with severe T cell defect. Clinical Infectious Diseases 2009; 49 (9): 1329-1338. doi: 10.1086/606059.
  • 11. Aghamohammadi A, Abolhassani H, Moazzami K, Parvaneh N, Rezaei N. Correlation between common variable immunodeficiency clinical phenotypes and parental consanguinity in children and adults. Journal of Investigational Allergology and Clinical Immunology 2010; 20 (5): 372-379. PMID: 20945602.
  • 12. Ardeniz O, Başoğlu OK, Gunşar F, Unsel M, Bayraktaroğlu S et al. Clinical and immunological analysis of 23 adult patients with common variable immunodeficiency. Journal of Investigational Allergology and Clinical Immunology 2010; 20 (3): 222-236. PMID: 20635788.
  • 13. Muşabak UH, Demirel F, Yeşillik S, Baysan A, Selçuk A et al. Adults with common variable immunodeficiency: a singlecentre experience. Turkish Journal of Medical Sciences 2017; 47 (1): 1-12. doi: 10.3906/sag-1503-22.
  • 14. Carvalho KI, Melo KM, Bruno FR, Snyder-Cappione JE, Nixon DF et al. Skewed distribution of circulating activated natural killer T (NKT) cells in patients with common variable immunodeficiency disorders (CVID). PLoS One 2010; 5 (9): e12652. doi: 10.1371/journal.pone.0012652.
  • 15. Kiaee F, Azizi G, Rafiemanesh H, Zainaldain H, Rizvi FS et al. Malignancy in common variable immunodeficiency: a systematic review and meta-analysis. Expert Review of Clinical Immunology 2019; 15 (10): 1105-1113. doi: 10.1080/1744666X.2019.1658523.
  • 16. Mellemkjaer L, Hammarstrom L, Andersen V, Yuen J, Heilmann C et al. Cancer risk among patients with IgA deficiency or common variable immunodeficiency and their relatives: a combined Danish and Swedish study. Clinical and Experimental Immunology 2002; 130 (3): 495-500. doi: 10.1046/j.1365-2249.2002.02004.x.
  • 17. Manesh AT, Azizi G, Heydari A, Kiaee F, Shaghaghi M et al. Epidemiology and pathophysiology of malignancy in common variable immunodeficiency? Allergologia et Immunopathologia 2017; 45 (6): 602-615. doi: 10.1016/j.aller.2017.01.006.
  • 18. Chapel H, Lucas M, Lee M, Bjorkander J, Webster D et al. Common variable immunodeficiency disorders: division into distinct clinical phenotypes. Blood 2008; 112 (2): 277-286. doi: 10.1182/blood-2007-11-124545.
  • 19. Wehr C, Kivioja T, Schmitt C, Ferry B, Witte T et al. The EUROclass trial: defining subgroups in common variable immunodeficiency. Blood 2008; 111 (1): 77-85. doi: 10.1182/blood-2007-06-091744.
  • 20. Ghoshal UC, Goel A, Ghoshal U, Jain M, Misra A et al. Chronic diarrhea and malabsorption due to hypogammaglobulinemia: a report on twelve patients. Indian Journal of Gastroenterology 2011; 30 (4): 170-174. doi: 10.1007/s12664-011-0111-y.
  • 21. Quinti I, Pulvirenti F, Milito C, Granata G, Giovannetti G et al. Haemolysis in patients with antibody deficiencies on immunoglobulin replacement treatment. Transfusion 2015; 55 (5): 1067-1074. doi: 10.1111/trf.12939.
  • 22. Salehzadeh M, Aghamohammadi A, Rezaei N. Evaluation of immunoglobulin levels and infection rate in patients with common variable immunodeficiency after immunoglobulin replacement therapy. Journal of Microbiology, Immunology and Infection 2010; 43 (1): 11-17. doi: 10.1016/S1684- 1182(10)60002-3.
  • 23. Lawrence MG, Palacios-Kibler TV, Workman LJ, Schuyler AJ, Steinke JW et al. Low serum IgE is a sensitive and specific marker for common variable immunodeficiency (CVID). Journal of Clinical Immunology 2018; 38 (3): 225-233. doi: 10.1007/s10875-018-0476-0.
  • 24. Thorarinsdottir K, Camponeschi A, Gjertsson I, Martensson IL. CD21-/low B cells: A snapshot of a unique B cell subset in health and disease. Scandinavian Journal of Immunology 2015; 82 (3): 254-261. doi: 10.1111/sji.12339.
  • 25. Agarwal S, Cunningham-Rundles C. Autoimmunity in common variable immunodeficiency. Current Allergy and Asthma Reports 2009; 9 (5): 347-352. doi: 10.1007/s11882-009- 0051-0.
Turkish Journal of Medical Sciences-Cover
  • ISSN: 1300-0144
  • Yayın Aralığı: Yılda 6 Sayı
  • Yayıncı: TÜBİTAK
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