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Reduced CD19 expression and decreased memory B cell numbers in transient hypogammaglobulinemia of infancy

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Abstract

We aimed to evaluate the role of the CD19 complex in the pathogenesis of transient hypogammaglobulinemia of infancy (THI) and to better characterize the subsets of memory B cells. The study population consisted of 22 male and 14 female patients with a mean age at presentation of 20 ± 9.9 months. The CD19 complex and B cell subsets were evaluated by flow cytometry. While the CD19 median fluorescence index (MFI) in patients with THI was significantly lower than controls (122.9 ± 66.7 in patients; 184.2 ± 39 in controls, p < 0.01), expression of CD21 and CD81 was increased (94.4 ± 3, 96.8 ± 2.5 % in patients; 91 ± 3.9; 94.7 ± 3.5 % in controls, p < 0.01 vs. p < 0.05, respectively). The expressions of switched memory B cells and IgM memory B cells were found to be reduced in THI. Considering that the CD19 complex regulates the events following antigen stimulation, the change in CD19 complex detected in THI may be related to insufficiency of antibody production.

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References

  1. Ochs HD, Stiehm ER, Winkelstein JA (2004) Antibody deficiencies. In: Stiehm ER, Ochs HD, Winkelstein JA (eds) Immunologic disorders in infants & children, 5th edn. WB Saunders, Philadelphia, pp 391–393

    Google Scholar 

  2. Fs Rosen, Cooper MD, Wedgewood JP (1984) The primary immunodeficiencies. N Engl J Med 311:300–310

    Article  Google Scholar 

  3. Siegel RL, Issekutz T, Schwaber J et al (1981) Deficiency of T helper cells in transient hypogammaglobulinemia of infancy. N Engl J Med 305:1307–1313

    Article  PubMed  CAS  Google Scholar 

  4. Dressler F, Peter HH, Muller W et al (1989) Transient hypogammaglobulinemia of infancy: five new cases, review of the literature and redefinition. Acta Paediatr Scand 78:767–773

    Article  PubMed  CAS  Google Scholar 

  5. Kowalczyk D, Mytar B, Zembala M (1997) Cytokine production in transient hypogammaglo-bulinemia and isolated IgA deficiency. J Allergy Clin Immunol 100:556–562

    Article  PubMed  CAS  Google Scholar 

  6. Kowalczyk D, Baran J, Webster ADB, Zembala M (2001) Intracellular cytokine production by Th1/Th2 lymphocytes and monocytes of children with symptomatic transient hypogammaglobulinemia of infancy (THI) and selective IgA deficiency (SIgAD). Clin Exp Immunol 127:507–512

    Article  Google Scholar 

  7. Dorsey MJ, Orange JS (2006) Impaired specific antibody response and increased B-cell population in transient hypogammaglobulinemia of infancy. Ann Allergy Asthma Immunol 97:590–595

    Article  PubMed  CAS  Google Scholar 

  8. Rudd CE (2006) Disabled receptor signaling and new primary immunodeficiency disorders. N Engl J Med 354:1874–1877

    Article  PubMed  CAS  Google Scholar 

  9. Cherukuri A, Cheng CP, Pierce SK (2001) The role of the CD19/CD21 complex in the B cell processing and presentation of complement-tagged antigens. J Immunol 167:163–172

    PubMed  CAS  Google Scholar 

  10. van Zelm MC, Reisli I, van der Burg M et al (2006) An antibody-deficiency syndrome due to mutations in the CD19 gene. N Engl J Med 354:1901–1912

    Article  PubMed  Google Scholar 

  11. Kanegane H, Agematsu K, Futatani T et al (2007) Novel mutations in a Japanese patient with CD19 deficiency. Genes Immun 8:663–670

    Article  PubMed  CAS  Google Scholar 

  12. Van Zelm MC, Smet J, Adams B et al (2010) CD81 gene defect in humans distrups CD19 complex formation and leads to antibody deficiency. J Clin Inves 120:1265–1274

    Article  Google Scholar 

  13. Thiel J, Kimmig L, Salzer U et al (2009) The clinical and immunological phenotype of human CD21 deficiency. Clin Immunol 131(supp 1):S57–S58

    Article  Google Scholar 

  14. Aksu G, Genel F, Koturoğlu G, Kurugöl Z, Kütükçüler N (2006) Serum immunoglobulin (IgG, IgM, IgA) and IgG subclass concentrations in healthy children: a study using nephelometric technique. Turk J Pediatr 48:19–24

    PubMed  Google Scholar 

  15. Stiehm ER (2008) The four most common pediatric immunodeficiencies. J Immunotoxicol 5:227–234

    Article  PubMed  Google Scholar 

  16. National Heart, Lung, and Blood Institute; National Institutes of Health. Global strategy for asthma management and prevention: Global Initative for Asthma (GINA): 2006 update (Internet). Available from:http//www.ginaasthma.org

  17. Bousquet J, Khaltaev N, Cruz AA et al (2008) Allergic rhinitis and its Impact on Asthma (ARIA) 2008 update (in collaboration with the World Health Organization GA(2)LEN and AllerGen). Allergy 86:8–160

    Article  Google Scholar 

  18. Böhme M, Svensson A, Kull I, Wahlgren CF (2000) Hanifin’s and Rajka’s minor criteria for atopic dermatitis: which do 2-year-olds exhibit? J Am Acad Dermatol 43:785–792

    Article  PubMed  Google Scholar 

  19. Driessen GJ, van Zelm MC, van Hagen PM et al (2011) B-cell replication history and somatic hypermutation status identify distinct pathophysiologic backgrounds in common variable immunodeficiency. Blood 118:6814–6823

    Article  PubMed  CAS  Google Scholar 

  20. Ikincioğullari A, Kendirli T, Doğu F, Eğin Y, Reisli I, Cin S, Babacan E (2004) Peripheral blood lymphocyte subsets in healthy Turkish children. Turk J Pediatr 46:125–130

    PubMed  Google Scholar 

  21. Carter RH, Fearon DT (1992) CD19:lowering the threshold for antigen receptor stimulation of B lymphocytes. Science 256:105–107

    Article  PubMed  CAS  Google Scholar 

  22. van Noesel CJ, Lankester AC, van Lier RA (1993) Dual antigen recognition by B cells. Immunol Today 14:8–11

    Article  PubMed  Google Scholar 

  23. Reisli I, Artac H, Pekcan S et al (2009) CD19 deficiency: a village screening study. Turk Arch Ped 44:127–130

    Google Scholar 

  24. Artac H, Reisli I, Kara R et al (2010) B-cell maturation and antibody responses in individuals carrying a mutated CD19 allele. Genes Immun 11:523–530

    Article  PubMed  CAS  Google Scholar 

  25. Poe JC, Hasegawa M, Tedder TF (2001) CD19, CD21, and CD22: multifaceted response regulators of B lymphocyte signal transduction. Intern Rev Immunol 20:739–762

    Article  CAS  Google Scholar 

  26. Ahearn JM, Fischer MB, Croix D et al (1996) Disruption of the Cr2 locus results in a reduction in B-1a cells and in an impaired B cell response to T-dependent antigen. Immunity 4:251–262

    Article  PubMed  CAS  Google Scholar 

  27. Sanyal M, Fernandez R, Levy S (2009) Enhanced B cell activation in the absence of CD81. Int Immunol 21:1225–1237

    Article  PubMed  CAS  Google Scholar 

  28. Gay-Gouy H, Bruhns z, Schmitt C et al (2000) The pseudo-immunoreceptor tyrosine-based activation motif of CD5 mediates its inhibitory action on B-cell receptor signaling. J Biol Chem 275:548–556

    Article  Google Scholar 

  29. Moschese V, Carsetti R, Graziani S et al (2007) Memory B-cell subsets as a predictive marker of outcome in hypogammaglobulinemia during infancy. J Allergy Clin Immunol 120:474–476

    Article  PubMed  CAS  Google Scholar 

  30. Bukowska-Straková K, Kowalczyk D, Baran J, Siedlar M, Kobylarz K, Zembala M (2009) The B-cell compartment in the peripheral blood of children with different types of primary immunodeficiency. Pediatr Res 66:28–34

    Article  PubMed  Google Scholar 

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Acknowledgments

This study was supported by Scientific Research Projects of Selcuk University (Project No: 08102019).

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Authors and Affiliations

  1. Department of Pediatric Immunology and Allergy, Selcuk University Selcuklu Medical Faculty, Alaeddin Keykubat Kampusu, 42075, Konya, Turkey

    Hasibe Artac & Reyhan Kara

  2. Department of Pediatric Immunology and Allergy, Necmettin Erbakan University Meram Medical Faculty, Konya, Turkey

    Bahar Gokturk & Ismail Reisli

Authors
  1. Hasibe Artac
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  2. Reyhan Kara
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  3. Bahar Gokturk
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  4. Ismail Reisli
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Correspondence to Hasibe Artac.

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Artac, H., Kara, R., Gokturk, B. et al. Reduced CD19 expression and decreased memory B cell numbers in transient hypogammaglobulinemia of infancy. Clin Exp Med 13, 257–263 (2013). https://doi.org/10.1007/s10238-012-0200-y

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  • DOI: https://doi.org/10.1007/s10238-012-0200-y

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