Skip to main content
SpringerLink
Log in

Hypogammaglobulinaemia: Recent Advances

  • Chapter
Immunodeficiency and Disease

Part of the book series: Immunology and Medicine Series ((IMME,volume 8))

Abstract

Soon after the technology for serum protein electrophoresis became available, Bruton was able to diagnose a child with hypogammaglobulinaemia who probably had acquired disease rather than the inherited X-linked variety1. Janewayet al. 2 described more cases a few years later, showing that adults can also be affected. The Medical Research Council’s survey in the 1960s showed that the majority of patients in the UK acquired the disease as adults3. A rough estimate at present is that there are about 500 patients with ‘primary’ non-familial hypogammaglobulinaemia in the UK population of approximately 55 million, with about 50 males having definite X-linked disease. The term ‘primary’ is taken to mean that the hypogammaglobulinaemia is not associated with a lymphoid malignancy (e.g. chronic lymphatic leukaemia, myeloma) or has been induced by drugs. However, this terminology is likely to be superseded by a more descriptive classification (see Table 2.1). Selective deficiencies of IgG subclasses and of IgA are discussed in Chapter 5. Some patients with selective deficiencies of both IgA and IgG2 may be included under the broad classification of ‘primary’ hypogammaglobulinaemia if the total serum IgG is low.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 39.99
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 54.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Bruton, O. C. (1952). Agammaglobulinaemia. Pediatrics, 9, 722

    PubMed  CAS  Google Scholar 

  2. Janeway, C. A., Apt, L. and Gitlin, D. (1953). Agammaglobulinaemia. Trans. Assoc. Am. Phys., 66, 200

    PubMed  CAS  Google Scholar 

  3. Hill, L. E. (1971). Clinical features of hypogammaglobulinaemia. In Hypogammaglobulinaemia in the United Kingdom. Medical Research Council Series SRS 310, p. 9

    Google Scholar 

  4. Asherson, G. L. and Webster, A. D. B. (1980). Diagnosis and treatment of immunodeficiency diseases, p. 7. (Oxford: Blackwell)

    Google Scholar 

  5. Kyong, C. U., Virella, D., Fudenberg, H. W. and Darby, C. P. (1978). X-linked immunodeficiency with increased IgM: clinical, ethnic and immunologic heterogeneity. Pediatr. Res., 12, 1024–6

    Article  PubMed  CAS  Google Scholar 

  6. Webster, A. D. B. (1984). Echovirus disease in hypogammaglobulinaemia patients. Clin. Rheumatic Dis., 10, 189–203

    CAS  Google Scholar 

  7. Bernatowska, E. and Madalinski, K. (1987). Intravenous immunoglobulin therapy in progressive encephalitis in X-linked hypogammaglobulinaemia. Acta Pediatr. Scand., 76, 155–3

    Article  CAS  Google Scholar 

  8. Erlendsson, K., Swartz, T. and Dwyer, J. M. (1985). Successful reversal of echovirus encephalitis in X-linked hypogammaglobulinaemia by intraventricular administration of immunoglobulin. N. Engl. J. Med., 312, 351–3

    Article  PubMed  CAS  Google Scholar 

  9. Roifman, C. M., Rao, C. P., Lederman, H. M., Lavi, S., Quinn, P. and Gelfand, E. W. (1986). Increased susceptibility to Mycoplasma infection in patients with hypogammaglobulinaemia. Am. J. Med., 80, 590–4

    Article  PubMed  CAS  Google Scholar 

  10. Taylor-Robinson, D. and McCormack, W. M. (1980). The genital mycoplasmas. N. Engl. J. Med., 302, 1003–10 and 1063–7

    Google Scholar 

  11. Webster, A. D. B., Furr, P. M., Hughes-Jones, N. C, Gorick, B. D. and Taylor-Robinson, D. (1988). Critical dependence on antibody for defence against mycoplasmas. Clin. Exp. Immunol. (In Press)

    Google Scholar 

  12. Taylor-Robinson, D., Furr P. M. and Webster, A. D. B. (1985). Ureaplasma urealyticum causing persistent urethritis in a patient with hypogammaglobulinaemia. Genitourin. Med., 61, 404–8

    PubMed  CAS  Google Scholar 

  13. The European Group for Immunodeficiencies (EGID) (1986). Intravenous gammaglobulin for immunodeficiency: report. Clin. Exp. Immunol., 65, 683–90

    Google Scholar 

  14. Prince, A. M., Piet, M. J. P. and Horowitz, B. (1986). Effect of Cohn fractionation conditions on infectivity of the AIDS virus. N. Engl. J. Med., 314, 386–7

    PubMed  CAS  Google Scholar 

  15. Roifman, C. M., Levison, H. and Gelfand, E. W. (1987). High dose versus low dose intravenous immunoglobulin in hypogammaglobulinaemia and chronic lung disease. Lancet, 1, 1075–7

    Article  PubMed  CAS  Google Scholar 

  16. Frøland, S. S. and Natvig, J. B. (1972). Surface-bound immunoglobulin on lymphocytes from normal and immunodeficient humans. Scand. J. Immunol., 1, 1

    Article  PubMed  Google Scholar 

  17. Golay, J. T. and Webster, A. D. B. (1986). B cells in patients with X-linked and ‘common variable’ hypogammaglobulinaemia. Clin. Exp. Immunol., 65, 100–4

    PubMed  CAS  Google Scholar 

  18. Fu, S. M., Hurley, J. N., McCune, J. M., Kunkel, H. G. and Good, R. A. (1980). Pre-B cells and other possible precursor lymphoid cell lines derived from patients with X-linked agammaglobulinaemia. J. Exp. Med., 152, 1519

    Article  PubMed  CAS  Google Scholar 

  19. Conley, M. E., Brown, P., Pickard, A. R., Buckley, R. N., Miller, D. S., Raskind, W. H., Singer, J. W. and Fialkow, P. J. (1986). Expression of the gene defect in X-linked agammaglobulinaemia. N. Engl. J. Med., 315, 564–7

    Article  PubMed  CAS  Google Scholar 

  20. Fearon, E. R., Winkelstein, J. A., Civin, C. I., Pardoll, D. M. and Vogelstein, B. (1987). Carrier detection in X-linked agammaglobulinaemia by analysis of X-chromosome inactivation. N. Engl. J. Med., 316, 427

    Article  PubMed  CAS  Google Scholar 

  21. Matamoros, N., Abad, E. and Webster, A. D. B. (1982). LDH isoenzymes in the T lymphocytes of patients with primary hypogammaglobulinaemia. Clin. Exp. Immunol., 50, 617

    PubMed  CAS  Google Scholar 

  22. Johnson, S. M., North, M. E., Asherson, G. L., Allsop, J., Watts, R. W. E. and Webster, A. D. B. (1977). Lymphocyte purine 5’nucleotidase deficiency in primary hypogammaglobulinaemia. Lancet, 1, 168

    Article  PubMed  CAS  Google Scholar 

  23. Seigal, F. R., Seigal, M. and Good, R. A. (1976). Suppression of B-cell differentiation by leucocytes from hypogammaglobulinaemic patients. J. Clin. Invest., 58, 109

    Article  Google Scholar 

  24. Rozynska, K. E., Spickett, G., Bryant, A., Webster, A. D. B. and Farrant, J. (1988). Cellular defects in immunodeficiency: a comparison between acquired hypogammaglobulinaemia and X-linked agammaglobulinaemia. In press

    Google Scholar 

  25. Kwan, S-P., Kunkel, L., Bruns, G., Wedgwood, R. J., Latt, S. and Rosen, F. S. (1986). Mapping of the X-linked agammaglobulinaemia locus by use of restriction fragment-length polymorphisms. J. Clin. Invest., 77, 649–52

    Article  PubMed  CAS  Google Scholar 

  26. Mensink, E. J. B. M., Thompson, A., Schot, J. D. L., Kraakman, M. E. M., Sandkuyl, L. and Schuurman, R. K. B. (1987). Genetic heterogeneity in X-linked agammaglobulinaemia complicates carrier detection and prenatal diagnosis. Clin. Genet. 31, 91–6

    Article  PubMed  CAS  Google Scholar 

  27. Malcolm, S., de Saint Basile, G., Arveiler, B., Lau, Y. L., Fischer, A., Griscelli, C., Debre, M., Mandel, J. L., Callard, R. E., Robertson, M. E., Goodship, J. A., Pembrey, M. E. and Levinsky, R. J. (1987). Close linkage of random DNA fragments from Xq21.3–22 to X-linked agammaglobulinaemia (XLA) Hum. Genet., 77, 172–4

    Article  PubMed  CAS  Google Scholar 

  28. Linch, D. C. and Levinsky, R. J. (1983). Prenatal diagnosis of foetal immunological disorders, Br. Med. Bull., 39, 399–404

    PubMed  CAS  Google Scholar 

  29. Purtilo, D. T., Cassel C. K., Young, J. P., Harper, R., Stephenson, S. R., Landing, B. H. and Jawter, G. F. (1975). X-linked recessive progressive combined variable immunodeficiency (Duncan’s disease). Lancet, 1, 935–40

    Article  PubMed  CAS  Google Scholar 

  30. Sakamoto, K., Freed, H. J. and Purtilo, D. T. (1980). Antibody responses to Epstein-Barr virus in families with the X-linked lymphoproliferative syndrome. J. Immunol., 125, 921–5

    PubMed  CAS  Google Scholar 

  31. Hanto, D. W., Frizzera, G., Gajl-Pecjalska, K. J. and Simmonds, R. C. (1985). Epstein-Barr virus, immunodeficiency and B cell lymphoproliferation. Transplantation, 39, 461–72

    Article  PubMed  CAS  Google Scholar 

  32. Harada, S., Bechtold, T., Seeley, J. K. and Purtilo, D. T. (1982). Cell-mediated immunity to Epstein-Barr virus (EBV) and natural killer (NK)-cell activity in the X-linked lymphoproliferative syndrome. Int. J. Cancer, 30, 739

    Article  PubMed  CAS  Google Scholar 

  33. Skare, J. C., Milunsky, A., Byron, K. S. and Sullivan, J. L. (1987). Mapping the X-linked lymphoproliferative syndrome. Proc. Natl. Acad. Sci., 84, 2015–18

    Article  PubMed  CAS  Google Scholar 

  34. Kinlen, L. J., Webster, A. D. B., Bird, A. G., Haile, R., Peto, J., Soothill, J. F. and Thompson, R. A. (1985). Prospective study of cancer in patients with hypogammaglobulinaemia. Lancet, 1, 263–6

    Article  PubMed  CAS  Google Scholar 

  35. Webster, A. D. B., Platts-Mills, T. A. E., Jannossy, G., Morgan, M. and Asherson, G. L. (1981). Autoimmune blood dyscrasias in five patients with hypogammaglobulinaemia: response of neutropenia to Vincristine. J. Clin. Immunol., 1, 113–18

    Article  PubMed  CAS  Google Scholar 

  36. Friedman, J. M., Fialkow, P. J., Davis, S. D., Ochs, H. D. and Wedgwood, R. J. (1977). Autoimmunity in the relatives of patients with immunodeficiency diseases. Clin. Exp. Immunol., 28, 375

    PubMed  CAS  Google Scholar 

  37. Kushner, D. S., Dubin, A., Donlon, W. P. and Bronsky, D. (1960). Familial hypogammaglobulinaemia, splenomegaly and leukopenia. Am. J. Med., 29, 33

    Article  PubMed  CAS  Google Scholar 

  38. Webster, A. D. B. and Asherson, G. L. (1974). Identification and function of T cells in the peripheral blood of patients with hypogammaglobulinaemia. Clin. Exp. Immunol., 18, 499

    PubMed  CAS  Google Scholar 

  39. Waldmann, T. A., Durm, M., Broder, S., Blackman, M., Blaese, R. M. and Strober, W. (1974). Role of suppressor cells in pathogenesis of common variable hypogammaglobulinaemia. Lancet, 2, 609

    Article  PubMed  CAS  Google Scholar 

  40. Platts-Mills, T. A. E., DeGast, G. C, Webster, A. D. B., Asherson, G. L. and Wilkins, S. R. (1981). Two immunologically distinct forms of late-onset hypogammaglobulinaemia. Clin. Exp. Immunol., 44, 383–8

    PubMed  CAS  Google Scholar 

  41. Ziegler-Heitbrock, H. W. L., Schramm, W., Stachel, P., Rumpold, H., Kraft, D., Wermcke, D., Von Der Helm, K., Eberle, J., Deinhardt, F., Rieber, E. P. and Riethmüller, G. (1985). Expansion of a minor subpopulation of peripheral blood lymphocytes (T8/Leu7+) in patients with haemophilia. Clin. Exp. Immunol., 61, 633–41

    PubMed  CAS  Google Scholar 

  42. De Gast, G., Wilkins, S. R., Webster, A. D. B., Rickinson, A. and Platts-Mills, T. A. E. (1980). Functional ‘immaturity’ of isolated B cells from patients with hypoglobulinaemia. Clin. Exp. Immunol., 42, 535

    PubMed  Google Scholar 

  43. Brenner, M. K., North, M. E., Chadda, H. R., Newton, C. A., Malkovsky, K., Webster, A. D. B. and Farrant, J. (1984). The role of B cell differentiation factors and specific T cell help in the pathogenesis of primary hypogammaglobulinaemia. Eur. J. Immunol., 14, 1021–7

    Article  PubMed  CAS  Google Scholar 

  44. Shah, T., Webster, A. D. B. and Peters, T. J. (1983). Lymphocyte enzyme activities in immunodeficiency syndromes with particular reference to common variable hypogammaglobulinaemia. Clin. Exp. Immunol., 53, 413–22

    PubMed  CAS  Google Scholar 

  45. Thompson, L. F., Saxon, A., O’Connor, R. D. and Fox, R. I. (1983). Ecto-5’-nucleotidase activity in human T cell subsets. J. Clin. Invest., 71, 892–4

    Article  PubMed  CAS  Google Scholar 

  46. Webster, A. D. B. (1978). Lymphocyte ecto 5’-nucleotidase deficiency. CIBA Foundation Symposium 68 (new series) p. 135. (Amsterdam: Excerpta Medica)

    Google Scholar 

  47. Shah, T., Farrant, J., Webster, A. D. B. and Peters, T. J. (1988). In vivo and in vitro immunoglobulin production after inhibition of lymphocyte ecto 5’-nucleotidase (submitted)

    Google Scholar 

  48. Shah, T., Webster, A. D. B. and Peters, T. J. (1984). Kinetic properties of 5’-nucleotidase in blood lymphocytes from healthy subjects, immunodeficient patients and cord blood. Clin. Exp. Immunol., 57, 149–54

    PubMed  CAS  Google Scholar 

  49. Mannhalter, J. W., Zlabinger, G. J. and Eibl, M. M. (1984). Defective macrophage-T cell interaction in common varied immunodeficiency. Prog. Immunodeficiency Res. Ther., 1, 147 (Elsevier)

    Google Scholar 

  50. Luquetti, A., Newton, C. A. and Webster, A. D. B. (1981). Cellular immunity in primary hypogammaglobulinaemia: evidence for a generalised lymphocyte defect in some patients with ‘common’variable hypogammaglobulinaemia. Allergol. et Immunopathol., 9, 295–306

    CAS  Google Scholar 

  51. Preud’Homme, J. L., Griscelli, C. and Seligmann, M. (1973). Immunoglobulins on the surface of lymphocytes in 50 patients with primary immunodeficiency diseases. Clin. Immunol. Immunopath., 1, 241

    Article  Google Scholar 

  52. Pereira, F. S., Webster, A. D. B. and Platts-Mills, T. A. E. (1982). Immature B cells in fetal development and immunodeficiency. Studies of IgM, IgG, Ig A and IgD production in vitro using Epstein-Barr virus activation. Eur. J. Immunol., 12, 540–6

    Article  PubMed  CAS  Google Scholar 

  53. Jeunet, F. J. and Good, R. A. (1968). Thymoma, immunologic deficiencies and hematological abnormalities. In Gergsma D. (ed.) Immunologic Deficiency Diseases in Man. (New York: The National Foundation, March of Dimes)

    Google Scholar 

  54. Hayward, A. R., Paolucci, P., Webster, A. D. B. and Kohler, P. (1982). Pre-B cell suppression by thymoma patient lymphocytes. Clin. Exp. Immunol., 48, 437–42

    PubMed  CAS  Google Scholar 

  55. Brenner, M. K., Reittie, J. G. C., Chadda, H. R., Pollock, A. and Asherson, G. L. (1984). Thymoma and hypogammaglobulinaemia with and without T-suppressor cells. Clin. Exp. Immunol., 58, 619–24

    PubMed  CAS  Google Scholar 

  56. Ho, M. (1984). Immunology of cytomegalovirus: immunosuppressive effects during infection. Birth defects, 20, 131–47

    PubMed  CAS  Google Scholar 

  57. Carney, W. P., Rubin, R. H., Hoffman, R. A., Hansen, W. P., Healey, K. and Hirsh, M. S. (1981). Analysis of T lymphocyte subsets in cytomegaloviral mononucleosis. J. Immunol. 126, 2114–16

    PubMed  CAS  Google Scholar 

  58. Gehrz, R. C., Christianson, W. R., Linner, K. M., Conroy, M. M., McCue, S. A. and Balfour, H. H. (1981). Cytomegalovirus — specific humoral and cellular immune responses in human pregnancy. J. Infect. Dis., 143, 391–5

    Article  PubMed  CAS  Google Scholar 

  59. Lane, H. C., Masur, H., Edgar, L. C., Whales, G., Rook, A. H. and Fauci, A. S. (1983). Abnormalities of B-cell activation and immunoregulation in patients with the acquired immunodeficiency syndrome. N. Engl J. Med., 309, 453

    Article  PubMed  CAS  Google Scholar 

  60. Schnittman, S. M., Lane, H. C., Higgins, S. E.. Folks, T. and Fauci, A. S. (1986). Direct polyclonal activation of human B lymphocytes by the acquired immune deficiency syndrome virus. Science, 233, 1084–6

    Article  PubMed  CAS  Google Scholar 

  61. Pahwa, R., Good, R. A. and Pahwa, S. (1987). Prematurity, hypogammaglobulinaemia and neuropathology with human immunodeficiency virus (HIV) infection. Proc. Natl. Acad. Sci. USA, 84, 3827–30

    Article  Google Scholar 

  62. Webster, A. D. B., Malkovsky, M., Patterson, S., North, M., Dalgleish, A. G., Beattie, R., Asherson, G. L. and Weiss, R. A. (1986). Isolation of retroviruses from two patients with ‘common variable’ hypogammaglobulinaemia. Lancet, 1, 581–3

    Article  PubMed  CAS  Google Scholar 

  63. Spickett, G. P., Millrain, M., North, M., Griffiths, J., Patterson, S. and Webster, A. D. B. (1988). Role of retroviruses in acquired hypogammaglobulinaemia. Clin. Exp. Immunol., submitted

    Google Scholar 

  64. Gendelman, H. E., Narayan, O., Molineauz, S., Clements, J. E. and Ghotbi, Z. (1985). Slow, persistent replication of lentiviruses: role of tissue macrophages and macrophage precursors in bone marrow. Proc. Natl. Acad. Sci. USA, 82, 7086–90

    Article  PubMed  CAS  Google Scholar 

  65. Chapel, H. M. and Bunch, C. (1988). Mechanisms of infection in chronic lymphocytic leukaemia. Sem. Haematol., 24, 291–6

    Google Scholar 

  66. Carter, A., Silvian, I., Tatoroky, I. and Spira, G. (1986). Impaired immunoglobulin synthesis in multiple myeloma: a B-cell dysfunction. Am. J. Hematol., 22, 143–54

    Article  PubMed  CAS  Google Scholar 

Download references

Authors
  1. A. D. B. Webster
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. G. P. Spickett
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Division of Immunological Medicine, Clinical Research Centre, Harrow,Middlesex, UK

    A. D. B. Webster (Consultant Physician and Senior Scientist) (Consultant Physician and Senior Scientist)

Rights and permissions

Reprints and permissions

Copyright information

© 1988 Kluwer Academic Publishers

About this chapter

Cite this chapter

Webster, A.D.B., Spickett, G.P. (1988). Hypogammaglobulinaemia: Recent Advances. In: Webster, A.D.B. (eds) Immunodeficiency and Disease. Immunology and Medicine Series, vol 8. Springer, Dordrecht. https://doi.org/10.1007/978-94-009-1275-5_2

Download citation

  • DOI: https://doi.org/10.1007/978-94-009-1275-5_2

  • Publisher Name: Springer, Dordrecht

  • Print ISBN: 978-94-010-7066-9

  • Online ISBN: 978-94-009-1275-5

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics

Search

Navigation