Inherited Dysgammaglobulinemia of Chickens

  • Albert A. Benedict
  • M. Eric Gershwin
  • Hans Abplanalp


Disorders of the immune system leading to hypogammaglobulinemia are considered to be either primary defects in stem cell precursors of B lymphocytes or disorders in the maturation of B cells. In the X-linked form of immunodeficiency B cells are usually absent; however, patients with the form of immunodeficiency referred to as varied immunodeficiency (VID) have normal or slightly below-normal numbers of circulating B cells (Dickler et al, 1974; Geha et al, 1974; Cooper et al, 1975). In VID, reduced immunoglobulin (Ig) results from defects that may occur at various stages of B-cell maturation—from impaired differentiation of pre-B cells to defective terminal differentiation (Pearl et al, 1979). The failure of B cells to mature into Ig-secreting cells has been associated with different abnormalities, such as excessive suppressor-cell activity (Waldmann et al, 1974, 1976; Siegal et al, 1976, 1978), defective helper-cell activity (De La Concha et al, 1977; Siegal et al, 1978), presence of serum and tissue-fluid inhibitors (Geha et al, 1974; Waldmann et al, 1976), and inherent B-cell defects (Geha et al, 1974; Ciccimarra et al, 1976; De La Concha et al, 1977). Clearly, VID is heterogeneous with respect to its phenotypic features, its mechanism of defective B-cell synthesis, and its inheritance patterns.


Peripheral Blood Lymphocyte Suppressor Cell Normal Chicken Normal Bird Normal Peripheral Blood Lymphocyte 
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  1. Benedict, A. A., Abplanalp, H. A., Pollard, L. W., and Tarn, L. Q., 1977, Immunodeficiency in chickens: A model for common variable hypogammaglobulinemia in man? Adv. Exp. Med. Biol. 88: 197.PubMedGoogle Scholar
  2. Benedict, A. A., Chanh, T. C., Tarn, L. Q., Pollard, L. W., Kubo, R. T., and Abplanalp, H. A., 1978, Inherited immunodeficiency in chickens, in: Animal Models of Comparative and Developmental Aspects of Immunity and Disease (M. E. Gershwin and E. L. Cooper, eds.), pp. 99–109, Pergamon Press, New York.Google Scholar
  3. Benedict, A. A., Tarn, L. Q., Pollard, L. W., Abplanalp, H. A., and Gershwin, M. E., 1980, Inherited 7S immunoglobulin deficiency in chickens: Late onset dysgammaglobulinemia and evaluation of the lymphocyte proliferative responses and other immunologic functions, Clin. Immunol. Immunopathol. 17: 1.PubMedCrossRefGoogle Scholar
  4. Blaese, R. M., Weiden, P. L., Koski, I., and Dooley, N., 1974, Infectious agammaglobulinemia: Transmission of immunodeficiency with grafts of agammaglobulinemic cells, J. Exp. Med. 140: 1097.PubMedCrossRefGoogle Scholar
  5. Blaese, R. M., Muchmore, A. V., Koski, I., and Dooley, N. J., 1977, Infectious agammaglobulinemia: Suppressor T cells with specificity for individual immuoglobulin classes, Adv. Exp. Med. Biol. 88: 155.PubMedGoogle Scholar
  6. Broom, B. C., De La Concha, E. G., Webster, D. B., Janossy, G. L., and Asherson, G. L., 1976, Intracellular immunoglobulin production in vitro by lymphocytes from patients with hypogammaglobulinemia and their effect on normal lymphocytes, Clin. Exp. Immunol. 23: 73.Google Scholar
  7. Brüggemann, J., Merkenschlager, M., Kirchner, B., and Lösch, U., 1967, Eine erblische Hypo-bzw. Dysgam-maglobulinämie beim Haushuhn, Naturwissenschaften 54: 97.CrossRefGoogle Scholar
  8. Brüggemann, J., Friedrich, B., and Lösch, U., 1969, Vergleichende Bestimmungen der Immunglobulin-Spiegel genuin dysgammaglobulinamischer und “hormoneil bursektomierter” Hühner, Z. Tierphysiol. Tierernaehr. Futtermittelkd. 24: 327.Google Scholar
  9. Calnek, B. W., and Witter, R. L., 1978, Marek’s disease, in: Diseases of Poultry (M. S. Hofstad, ed.), pp. 385–417, Iowa State University Press, Ames.Google Scholar
  10. Chanh, T. C., Benedict, A. A., Tarn, L. Q., Abplanalp, H., and Gershwin, M. E., 1980, Inherited 7S immunoglobulin deficiency in chickens: Presence of suppressor T cells which suppress synthesis of 7S immunoglobulin but not IgM, J. Immunol. 125: 108.PubMedGoogle Scholar
  11. Ciccimarra, F., Rosen, F. S., and Schneeberger, E., 1976, Failure of heavy chain glycosylation of IgG in some patients with common variable agammaglobulinemia, J. Clin, Invest. 57: 1386.CrossRefGoogle Scholar
  12. Cooper, M. D., Keightley, R. G., and Lawton, A. R., 1975, Defective T and B cells in primary immunodeficiency, in: Membrane Receptors of Lymphocytes (M. Seligmann, J. L. Preud’homme, and F. M. Kourilsk, eds.), pp. 431–442, North-Holland, Amsterdam.Google Scholar
  13. De La Concha, E. G., Oldman, G., Webster, A. D. B., Asherson, G. L., and Platts-Mills, T. A. E., 1977, Quantitative measurements of T and B cell function in “variable” primary hypogammaglobulinaemia: Evidence for a consistent B-cell defect, Clin. Exp. Immunol. 27: 208.Google Scholar
  14. Dickler, H. B., Adkinson, N. F., Jr., Fisher, R. I., and Terry, W. D., 1974, Lymphocytes in patients with variable immunodeficiency and panhypogammaglobulinemia, J. Clin. Invest. 53: 834.PubMedCrossRefGoogle Scholar
  15. Droege, W., 1971, Amplifying and suppressive effect of thymus cells, Nature (London) 234: 549.CrossRefGoogle Scholar
  16. Droege, W., 1975, Immunosuppressive effect of syngeneic thymus cells on allograft rejection, Proc. Natl. Acad. Sci. USA 72: 2371.PubMedCrossRefGoogle Scholar
  17. Droege W., 1976, The antigen-inexperienced thymic suppressor cells: A class of lymphocytes in the young chicken thymus that inhibits antibody production and cell-mediated immune responses, Eur. J. Immunol. 6: 279.PubMedCrossRefGoogle Scholar
  18. Fudenberg, H. H., 1966, Immunologic deficiency, autoimmune disease, and lymphoma: Observations, implications, and speculations, Arthritis Rheum. 9: 464.PubMedCrossRefGoogle Scholar
  19. Fudenberg, H. H., 1971, Genetically determined immune deficiency as the predisposing cause of “autoimmunity” and lymphoid neoplasia, Am. J. Med. 51: 295.PubMedCrossRefGoogle Scholar
  20. Fudenberg, H. H., German, J. L., and Kinkel, H. G., 1962, The occurrence of rheumatoid factor and other abnormalities in families of patients with agammaglobulinemia, Arthritis Rheum. 5: 565.PubMedCrossRefGoogle Scholar
  21. Geha, R. S., Schneeberger, E., Merler, E., and Rosen, F. S., 1974, Heterogeneity of “acquired” or common variable agammaglobulinemia, N. Engl. J. Med. 291: 1.PubMedCrossRefGoogle Scholar
  22. Gershwin, M. E., and Steinberg, A. D., 1975, The pathogenetic basis of animal and human autoimmune disease, Clin. Immunol. Immunopathol. 5: 38.CrossRefGoogle Scholar
  23. Gershwin, M. E., Erickson, K., Montero, J., Abplanalp, H., Eklund, J., Benedict, A. A., and Ikeda, R. M., 1980, The immunopathology of spontaneous acquired dysgammaglobulinemia of chickens, Clin. Immunol. Immunopathol. 17: 15.PubMedCrossRefGoogle Scholar
  24. Good, R. A., Rothstein, J., and Mazzitello, W. F., 1957, The simultaneous occurrence of rheumatoid arthritis and agammaglobulinemia, J. Lab. Clin. Med. 49: 343.PubMedGoogle Scholar
  25. Grebenau, M. D., and Thorbecke, G. J., 1978, T cell tolerance in the chicken. I. Parameters affecting tolerance induction to human γ-globulin in agammaglobulinemic and normal chickens, J. Immunol. 120: 1046.PubMedGoogle Scholar
  26. Grebenau, M. D., Lerman, S. P., Palladino, M. A., and Thorbecke, G. J., 1976, Suppression of adoptive antibody responses by addition of spleen cells from agammaglobulinaemic chickens “immunised” with histocompatible bursa cells, Nature (London) 260: 46.CrossRefGoogle Scholar
  27. Janeway, C. A., Gitlin, D., Craig, J. M., and Grice, D. S., 1956, “Collagen disease” in patients with congenital agammaglobulinemia, Trans. Assoc. Am. Physicians 69: 93.PubMedGoogle Scholar
  28. Kermani-Arab., V., and Leslie, G. A., 1977, Suppression of immunoglobulin synthesis by transplantation of T-cells from anti-μ bursectomized chickens into normal recipients, J. Immunol. 119: 530.PubMedGoogle Scholar
  29. Lösch, U., and Hoffman-Fezer, G., 1973, Vergleichende histologische und immunologische Untersuchungen an immunologisch defekten Huehnern. II. Genuin dysgammaglobulinaemische Huehner, Zentralbl. Veterinaermed. Reihe A 20: 596.CrossRefGoogle Scholar
  30. Lösch, U., Friedrich, B., and Hoffman-Fezer, G., 1971, Zur Charakterisierung eines erblichen Immundefktes bein Haushuhn, Tieraerztl. Umsch. 26: 338.Google Scholar
  31. Montero, J., Gershwin, M. E., Eklund, J., Abplanalp, H., Erickson, K., Tarn, L., Benedict, A. A., and Ikeda, R. M., 1979, Autoimmune manifestations of selective 7S immunoglobulin deficiency of chickens, Clin. Immunol. Immunopathol. 14: 334.PubMedCrossRefGoogle Scholar
  32. Payne, L. N., 1971, The lymphoid system, in: Physiology and Biochemistry of the Domestic Fowl (D. J. Bell and B. M. Freeman, eds.), pp. 985–1037, Academic Press, New York.Google Scholar
  33. Pearl, E. R., Lawton, A. R., and Cooper, M. D., 1979, Informative defects of B lymphocyte differentiation in human antibody deficiency disorders, in: B Lymphocytes in the Immune Response (M. Cooper, D. E. Mosier, I. Scher, and E. S. Vitetta, eds.), pp. 341–348, Elsevier/North-Holland, New York.Google Scholar
  34. Rose, N. R., Bacon, L. D., Sundick, R. S., Kong, Y. M., Esquivel, P., and Bigazzi, P. E., 1977, Genetic regulation in autoimmune thyroiditis, in: Autoimmunity (N. Talal, ed.), pp. 63–87, Academic Press, New York.Google Scholar
  35. Schwartz, S. A., Choi, Y. S., Shou, L., and Good, R. A., 1977, Modulatory effects on immunoglobulin synthesis and secretion by lymphocytes from immunodeficient patients, J. Clin. Invest. 59: 1176.PubMedCrossRefGoogle Scholar
  36. Siegal, F. P., Siegal, M., and Good, R. A., 1976, Suppression of B cell differentiation by leukocytes from hypogammaglobulinemic patients, J. Clin. Invest. 58: 109.PubMedCrossRefGoogle Scholar
  37. Siegal, F. P., Siegal, M., and Good, R. A., 1978, Role of helper, suppressor and B-cell defects in the pathogenesis of the hypogammaglobulinemias, N. Engl. J. Med. 299: 172.PubMedCrossRefGoogle Scholar
  38. Tarn, L. Q., and Benedict, A. A., 1975, Elevated 7S immunoglobulin and acute phase proteins in adjuvant-injected chickens, Proc. Soc. Exp. Biol. Med. 150: 340.Google Scholar
  39. Toivanen, A., Eskola, J., and Toivanen, P., 1976, Restorative effects of different embryonic cells transplanted into immunodeficient chick embryos, Ann. Immunol. (Inst. Pasteur) 127C: 923.Google Scholar
  40. Waldmann, T. A., and Broder, M. D., 1977, Suppressor cells in the regulation of the immune response, Prog. Clin. Immunol. 3: 155.PubMedGoogle Scholar
  41. Waldmann, T. A., Broder, S., Blaese, R. M., Durm, M., Blackman, M., and Strober, W., 1974, Role of suppressor T cells in pathogenesis of common variable hypogammaglobulinemia, Lancet 2: 609.PubMedCrossRefGoogle Scholar
  42. Waldmann, T. A., Broder, S., Krakauer, R., McDermott, R. P., Durm, M., Goldman, C., and Meade, B., 1976, The role of suppressor cells in the pathogenesis of common variable hypogammaglobulinemia and the immunodeficiency associated with myeloma, Fed. Proc. Fed. Am. Soc. Exp. Biol. 35: 2067.Google Scholar
  43. Warner, N. L., 1977, Genetic aspects of autoimmune disease in animals, in: Autoimmunity (N. Talal, ed.), pp. 33–62, Academic Press, New York.Google Scholar
  44. Yamaga, K., and Benedict, A. A., 1975, Class, amounts and affinities of anti-dinitrophenyl antibodies in chickens. I. Production of 7S and 17S antibodies of equal affinity by intravenous injection of antigen, J. Immunol. 115: 750.PubMedGoogle Scholar

Copyright information

© Plenum Press, New York 1981

Authors and Affiliations

  • Albert A. Benedict
    • 1
  • M. Eric Gershwin
    • 2
  • Hans Abplanalp
    • 3
  1. 1.Department of MicrobiologyUniversity of HawaiiHonoluluUSA
  2. 2.Section of Rheumatology-Clinical ImmunologySchool of Medicine, University of CaliforniaDavisUSA
  3. 3.Department of Avian SciencesUniversity of CaliforniaDavisUSA

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