Summary
Humoral and cell-mediated immune reactions of a 36-year old patient with a late onset of an agammaglobulinaemia and of his immediate relatives have been investigated. The immunoglobulins gamma-G, gamma-A, gamma-M, gamma-E and gamma-D, and plasma cells in the bone marrow could not be detected in the patient's serum. Immune reactions with PPD and tetanus toxoid as well as the stimulation of the lymphocytes with PHA indicate that the T-cell system was intact. The examination of the peripheral blood lymphocytes by immunofluorescence techniques gave a normal number of B-lymphocytes. These findings could be corroborated by the results of lymphocyte stimulations with anti-IgG. The data suggest that the maturation of the B-lymphocytes to plasma cells may be disturbed.
Zusammenfassung
Bei einem 36jährigen Patienten mit einer kompletten Agammaglobulinämie und seinen Verwandten wurden humorale und celluläre Immunität untersucht. Im Knochenmark des Patienten fehlten die Plasmazellen. Sowohl die positiven Hautreaktionen gegenüber PPD und Tetanustoxoid als auch die Stimulation der peripheren Lymphocyten mit PHA deuten auf ein intaktes T-Zellsystem hin. Der Anteil der B-Lymphocyten im peripheren Blut war normal. Dieser Befund korreliert mit einer normalen Stimulationsrate mit Anti-IgG-Serum. Die Befunde sprechen für eine Reifungsstörung der B-Lymphocyten zu Plasmazellen.
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Literatur
Alm, G. v., Peterson, R.D.A.: Antibody and immunoglobulin production at the cellular level in bursectomized irradiated chickens. J. exp. Med.129, 1247 (1969)
Bankhurst, A.D., Warner, N.L.: Surface immunoglobulins on mouse lymphoid cells. J. Immunology107, 368 (1971)
Bruton, O.C.: Agammaglobulinaemia. Pediatrics9, 722 (1952)
Claman, H.N.: Human thymus cell cultures-evidence for two functional populations. Proc. Soc. exp. Biol. (N.Y.)121, 236 (1966)
Cooper, M.D., Lawton, A.R.: Circulating B-cells in patients with immunodeficiency. Amer. J. Path.69, 513 (1972)
Cooper, M.D., Lawton, A.R., Bockman, D.E.: Agammaglobulinaemia with B-lymphocytes. Specific defect of plasma-cell differentiation. Lancet1971 II 791
Doenhoff, M.U., Davies, A.J.S., Leuchars, E., Wallis, V.: The thymus and circulating lymphocytes of mice. Proc. roy. Soc. B176, 69 (1971)
Good, R.A., Martinez, C., Gabrielsen, A.E.: Clinical considerations of the thymus in immunobiology. In: The thymus in immunobiology. Ed. by R.A. Good and A.E. Gabrielsen, p. 5. New York: Harper & Row 1964
Janossy, G., Greaves, M.F.: II. Discriminating stimulation of lymphocyte subpopulations by phytomitogens and heterologous antilymphocyte sera. Clin. exp. Immunol.10, 525 (1972)
Johansson, B., Klein, E.: Cell surface localized IgM-Kappa immunoglobulin reactivity in a case of chronic lymphocytic leukaemia. Clin. exp. Immunol.6, 421 (1970)
Jones, G., Torrigiani, R., Roitt, I.M.: Immunoglobulin determinants on mouse lymphocytes. J. Immunol.106, 1425 (1971)
Kincade, P.W., Lawton, A.R., Cooper, M.D.: Restriction of surface immunoglobulin determinants to lymphocytes of the plasma cell line. J. Immunol.106, 1421 (1971)
Kissmeyer-Nielsen, F., Thorsby, E.: Human transplantation antigens. Transplant. Rev. No. 4, p. 121. Stockholm: Göran Möller 1970
Mancini, G., Carbonara, A.O., Heremans, J.F.: Immunochemical quantitation of antigens by single radial immunodiffusion. Immunochemistry2, 235 (1965)
Oppenheim, J.J., Rogentine, G.N., Terry, W.D.: The transformation of human lymphocytes by monkey antisera to human immunoglobulins. Immunology16, 123 (1969)
Rabellino, E., Colon, S., Grey, H.M., Unanue, E.R.: Immunoglobulins on the surface of lymphocytes. I. Distribution and quantitation. J. exp. Med.133, 156 (1971)
Raff, M.F.: Two distinct populations of peripheral lymphocytes in mice distinguishable by immunofluoresence. Immunology19, 637 (1970)
Scheidegger, J.J.: Une micro méthode de l'immunoélectrophorése. Int. Arch. Allergy7, 103 (1955)
Sell, S., Gell, P.G.H.: Studies on rabbit lymphocytes in vitro. I. Stimulation of blast transformation with an antiallotype serum. J. exp. Med.122, 423 (1965)
Walters, C.S., Wigzell, H.: Demonstration of heavy and light chain antigenic determinants on the cell-bound receptor for antigen. Similiarities between membraneattached and humoral antibodies produced by the same cell. J. exp. Med.132, 1233 (1970)
Wilson, J.D., Nossal, G.J.V.: Identification of human T- and B-lymphocytes in normal peripheral blood and in chronic lymphocytic leucaemia. Lancet1971 II 788
Wolf, J.K., Gökcen, M., Good, R.A.: Hereto-familial disease of the mesenchymal tissues: Clinical and laboratory study of one family. J. Lab. clin. Med.61, 230 (1963)
Wollheim, F.A., Belfrage, S., Cöster, C., Lindholm, H.: Primary “acquired” hypogammaglobulinemia: clinical and genetic aspects of nine cases. Acta med. scand.176, 1 (1964)
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This work was supported by the Deutsche Forschungsgemeinschaft Bad Godesberg.
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Hopf, U., Knolle, J., Bauchinger, M. et al. A case of agammaglobulinemia characterized by a defect of B-lymphocyte-differentiation to plasma cells. Klin Wochenschr 52, 527–530 (1974). https://doi.org/10.1007/BF01468723
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DOI: https://doi.org/10.1007/BF01468723