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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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