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. 1989 Oct;78(1):1–6.

Accessory and T cell defects in acquired and inherited hypogammaglobulinaemia.

K E Rozynska 1, G P Spickett 1, M Millrain 1, A Edwards 1, A Bryant 1, A D Webster 1, J Farrant 1
PMCID: PMC1534610  PMID: 2530014

Abstract

Cellular defects in patients with common variable hypogammaglobulinaemia (CVH) and X-linked agammaglobulinaemia (XLA) have been studied in vitro, using a mitogen-driven system of immunoglobulin production. We have confirmed our previous finding of impaired low-density (dendritic) accessory cell function in CVH and now show that accessory cell function is normal in XLA. We demonstrate that macrophage accessory function is normal in CVH. T cell help for IgM production is also deficient in CVH, and T cell help in XLA is also abnormal for both IgG and IgM. Some XLA patients have excessive T suppressor activity. The contribution of these defects to the clinical states is discussed.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Ashman R. F., Saxon A., Stevens R. H. Profile of multiple lymphocyte functional defects in acquired hypogrammaglobulinemia, derived from in vitro cell recombination analysis. J Allergy Clin Immunol. 1980 Apr;65(4):242–256. doi: 10.1016/0091-6749(80)90151-7. [DOI] [PubMed] [Google Scholar]
  2. Brenner M. K., North M. E., Chadda H. R., Newton C. A., Malkovsky M., Webster A. D., Farrant J. The role of B cell differentiation factors and specific T cell help in the pathogenesis of primary hypogammaglobulinemia. Eur J Immunol. 1984 Nov;14(11):1021–1027. doi: 10.1002/eji.1830141111. [DOI] [PubMed] [Google Scholar]
  3. De Gast G. C., Wilkins S. R., Webster A. D., Rickinson A., Platts-Mills T. A. Functional 'immaturity' of isolated B cells from patients with hypogammaglobulinaemia. Clin Exp Immunol. 1980 Dec;42(3):535–544. [PMC free article] [PubMed] [Google Scholar]
  4. Dosch H. M., Gelfand E. W. Functional differentiation of B lymphocytes in agammaglobulinemia. III. Characterization of spontaneous suppressor cell activity. J Immunol. 1978 Nov;121(5):2097–2105. [PubMed] [Google Scholar]
  5. Eibl M. M., Mannhalter J. W., Zielinski C. C., Ahmad R. Defective macrophage--T-cell interaction in common varied immunodeficiency. Clin Immunol Immunopathol. 1982 Mar;22(3):316–322. doi: 10.1016/0090-1229(82)90048-4. [DOI] [PubMed] [Google Scholar]
  6. Eibl M. M., Mannhalter J. W., Zlabinger G., Mayr W. R., Tilz G. P., Ahmad R., Zielinski C. C. Defective macrophage function in a patient with common variable immunodeficiency. N Engl J Med. 1982 Sep 23;307(13):803–806. doi: 10.1056/NEJM198209233071307. [DOI] [PubMed] [Google Scholar]
  7. Farrant J., Bryant A. E., Lever A. M., Edwards A. J., Knight S. C., Webster A. D. Defective low-density cells of dendritic morphology from the blood of patients with common variable hypogammaglobulinaemia: low immunoglobulin production on stimulation of normal B cells. Clin Exp Immunol. 1985 Jul;61(1):189–194. [PMC free article] [PubMed] [Google Scholar]
  8. Farrant J., Bryant A., Almandoz F., Spickett G., Evans S. W., Webster A. D. B cell function in acquired "common-variable" hypogammaglobulinemia: proliferative responses to lymphokines. Clin Immunol Immunopathol. 1989 May;51(2):196–204. doi: 10.1016/0090-1229(89)90019-6. [DOI] [PubMed] [Google Scholar]
  9. Farrant J., Newton C. A., North M. E., Weyman C., Brenner M. K. Production of antibody by human B cells under serum-free conditions. J Immunol Methods. 1984 Mar 30;68(1-2):25–34. doi: 10.1016/0022-1759(84)90133-9. [DOI] [PubMed] [Google Scholar]
  10. Fernandez L. A., MacSween J. M. The suppressive effects of monocytes in the autologous mixed lymphocyte reaction. Immunology. 1981 Dec;44(4):653–659. [PMC free article] [PubMed] [Google Scholar]
  11. Fiedler W., Sykora K. W., Welte K., Kolitz J. E., Cunningham-Rundles C., Holloway K., Miller G. A., Souza L., Mertelsmann R. T-cell activation defect in common variable immunodeficiency: restoration by phorbol myristate acetate (PMA) or allogeneic macrophages. Clin Immunol Immunopathol. 1987 Aug;44(2):206–218. doi: 10.1016/0090-1229(87)90066-3. [DOI] [PubMed] [Google Scholar]
  12. Knight S. C., Balfour B. M., O'Brien J., Buttifant L., Sumerska T., Clarke J. Role of veiled cells in lymphocyte activation. Eur J Immunol. 1982 Dec;12(12):1057–1060. doi: 10.1002/eji.1830121214. [DOI] [PubMed] [Google Scholar]
  13. Knight S. C., Farrant J., Bryant A., Edwards A. J., Burman S., Lever A., Clarke J., Webster A. D. Non-adherent, low-density cells from human peripheral blood contain dendritic cells and monocytes, both with veiled morphology. Immunology. 1986 Apr;57(4):595–603. [PMC free article] [PubMed] [Google Scholar]
  14. Matamoros N., Abad E., Webster A. D. LDH isoenzymes in the T lymphocytes of patients with primary hypogammaglobulinaemia. Clin Exp Immunol. 1982 Dec;50(3):617–620. [PMC free article] [PubMed] [Google Scholar]
  15. North M. E., Spickett G. P., Allsop J., Webster A. D., Farrant J. Defective DNA synthesis by T cells in acquired 'common-variable' hypogammaglobulinaemia on stimulation with mitogens. Clin Exp Immunol. 1989 Apr;76(1):19–23. [PMC free article] [PubMed] [Google Scholar]
  16. Nussenzweig M. C., Steinman R. M., Gutchinov B., Cohn Z. A. Dendritic cells are accessory cells for the development of anti-trinitrophenyl cytotoxic T lymphocytes. J Exp Med. 1980 Oct 1;152(4):1070–1084. doi: 10.1084/jem.152.4.1070. [DOI] [PMC free article] [PubMed] [Google Scholar]
  17. Pearl E. R., Vogler L. B., Okos A. J., Crist W. M., Lawton A. R., 3rd, Cooper M. D. B lymphocyte precursors in human bone marrow: an analysis of normal individuals and patients with antibody-deficiency states. J Immunol. 1978 Apr;120(4):1169–1175. [PubMed] [Google Scholar]
  18. Platts-Mills T. A., De Gast G. C., Webster A. D., Asherson G. L., Wilkins S. R. Two immunologically distinct forms of late-onset hypogammaglobulinaemia. Clin Exp Immunol. 1981 May;44(2):383–388. [PMC free article] [PubMed] [Google Scholar]
  19. Siegal F. P., Siegal M., Good R. A. Suppression of B-cell differentiation by leukocytes from hypogammaglobulinemic patients. J Clin Invest. 1976 Jul;58(1):109–122. doi: 10.1172/JCI108439. [DOI] [PMC free article] [PubMed] [Google Scholar]
  20. Smith C. I., Hammarström L., Lindahl M., Lockner D. Kinetics of the spontaneously occurring common variable hypogammaglobulinemia: an analysis of two individuals with previously normal immunoglobulin levels. Clin Immunol Immunopathol. 1985 Oct;37(1):22–29. doi: 10.1016/0090-1229(85)90131-x. [DOI] [PubMed] [Google Scholar]
  21. Spickett G. P., Millrain M., Beattie R., North M., Griffiths J., Patterson S., Webster A. D. Role of retroviruses in acquired hypogammaglobulinaemia. Clin Exp Immunol. 1988 Nov;74(2):177–181. [PMC free article] [PubMed] [Google Scholar]
  22. Tedder T. F., Crain M. J., Kubagawa H., Clement L. T., Cooper M. D. Evaluation of lymphocyte differentiation in primary and secondary immunodeficiency diseases. J Immunol. 1985 Sep;135(3):1786–1791. [PubMed] [Google Scholar]
  23. Van Voorhis W. C., Witmer M. D., Steinman R. M. The phenotype of dendritic cells and macrophages. Fed Proc. 1983 Nov;42(14):3114–3118. [PubMed] [Google Scholar]
  24. Waldmann T. A., Durm M., Broder S., Blackman M., Blaese R. M., Strober W. Role of suppressor T cells in pathogenesis of common variable hypogammaglobulinaemia. Lancet. 1974 Sep 14;2(7881):609–613. doi: 10.1016/s0140-6736(74)91940-0. [DOI] [PubMed] [Google Scholar]
  25. Webster A. D., Dalgleish A. G., Malkovsky M., Beattie R., Patterson S., Asherson G. L., North M., Weiss R. A. Isolation of retroviruses from two patients with "common variable" hypogammaglobulinaemia. Lancet. 1986 Mar 15;1(8481):581–583. doi: 10.1016/s0140-6736(86)92809-6. [DOI] [PubMed] [Google Scholar]
  26. de la Concha E. G., Oldham G., Webster A. D., Asherson G. L., Platts-Mills T. A. Quantitative measurements of T- and B-cell function in "variable" primary hypogammaglobulinaemia: evidence for a consistent B-cell defect. Clin Exp Immunol. 1977 Feb;27(2):208–215. [PMC free article] [PubMed] [Google Scholar]

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