Summary
The T- and B-lymphocytes of 17 patients with immunodeficiency diseases were determined quantitatively. Simultaneously, their functional behavior, i.e. the response to stimulation by phytohemagglutinin and the immunoglobulin levels were studied.
In a preliminary study devised to check the accuracy of the methods used, the cell markers (rosette formation with sheep erythrocytes for T-cells and membrane immunofluorescence for B-cells) were defined by means of two different lymphoid cell populations: as nearly pure T-cells the fresh suspensions prepared from thymic biopsies of 3 young children were used, as B-cell rich preparations the isolated lymphocytes of 3 patients with chronic lymphatic leukemia.
Immunodeficiency syndromes were classified according to the recommendations of a World Health Organization expert committee [12, 13]: 2 patients with agammaglobulinemia, 5 with variable immunodeficiency, 4 with ataxia teleangiectatica, 3 with severe combined immunodeficiency and 3 with Wiskott-Aldrich syndrome were extensively studied (Table 2). The results are relevant for the better understanding of normal differentiation and its disturbances leading to the different deficiency syndromes (cf. Fig. 1).
Zusammenfassung
Bei 17 Patienten mit angeborenen Immunmangelkrankheiten wurden die T- und B-Lymphocyten quantitativ untersucht. Gleichzeitig wurden zur funktionellen Beurteilung der beiden Zellpopulationen die Antwort auf Stimulation mittels Phytohämagglutinin und die Serumkonzentration der Immunglobuline untersucht.
In einer vorbereitenden Studie wurde die Zuverlässigkeit der verwendeten Methoden kontrolliert; dafür wurden die Zellmarker (Rosettenbildung mit Schafserythrocyten für T-Zellen, Membranimmunfluorescenz für B-Zellen) anhand von zwei unterschiedlichen Lymphoidzellpopulationen genauer definiert: als nahezu reine T-Zellen wurden frisch zubereitete Suspensionen aus Thymusbiopsien von drei jungen Kindern verwendet, als B-zellreiche Präparate die isolierten Lymphocyten von drei Patienten mit chronisch lymphatischer Leukämie.
Zur Klassifizierung der angeborenen Immunmangelsyndrome wurden die Vorschläge eines Expertenkomitees der Weltgesundheitsorganisation befolgt [12, 13]: 2 Patienten mit Agammaglobulinämie, 5 mit variablem Immunmangel, 4 mit Ataxia telcangiectatica, 3 mit schwerem kombiniertem Immunmangel und 3 mit Wiskott-Aldrich-Syndrom konnten ausführlich untersucht werden (Ergebnisse s. Tabelle 2). Die kritische Beurteilung der Resultate trägt zum besseren Verständnis der normalen Differenzierung lymphoider Zellen und der zur Immunmangelkrankheit führenden Störungen bei (vgl. dazu Abb. 1).
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Prof. Windorfer to his 65th birthday.
This study was supported by a grant of the “Schweiz. Nationalfonds zur Förderung der wissenschaftlichen Forschung” Nr. 32068 Z.
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Schwenk, H.U., Gimpert, E., Plüss, H.J. et al. Lymphocyte markers for B- and T-cells in primary immunodeficiency diseases. Klin Wochenschr 52, 426–432 (1974). https://doi.org/10.1007/BF01468583
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DOI: https://doi.org/10.1007/BF01468583