Abstract
Ten patients with common variable immunodeficiency were classified into three groups according to the number of circulating B-cells, i.e. B-cells being absent (three patients), very low (three patients) or within the normal range (four patients). The four patients in the last group showed significant proliferative responses to the T-independent B-cell mitogen, formalin-fixed Staphylococcus aureus, Cowan I. Further study of these patients by co-cultures with allogeneic T or B-cells in various combinations with pokeweed mitogen showed that two patients had an intrinsic B-cell defect without T-cell defect. The third patient had a T-cell dysfunction (i.e. his T-cell could only help the B-cells of some individuals) resulting in a defect in Ig production. The T-cells of the fourth patient showed poor helper function towards all controls. All six patients with absent or very low numbers of B-cells in group I and II had normal T-cell helper function. This study demonstrates that the immunological defect in common variable immunodeficiency is most often a B-cell defect at different stages of their differentiation with sometimes an additional T-cell dysfunction.
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Abbreviations
- Con A:
-
concanavalin A
- CVID:
-
common variable immunodeficiency
- E:
-
erythrocyte
- EAC:
-
erythrocyte-antibody-complement
- FCS:
-
fetal calf serum
- HEPES:
-
N′-2-Hydroxyethylpiperazine-N′-Ethanesulphonic Acid
- Ig:
-
immunoglobulin
- PBL:
-
peripheral blood lymphocyte
- PHA:
-
phytohaemagglutinin
- PWM:
-
pokeweed mitogen
- RFC:
-
rosette-forming cells
- RPMI:
-
Roswell Park Memorial Institute
- Staph A:
-
formalin-fixed Staphylococcus aureus, Cowan I
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Matsuoka, H., Okada, J., Takahashi, T. et al. In vitro analysis of lymphocyte functions in common variable immunodeficiency: heterogeneity in B-cell defects. Eur J Pediatr 145, 252–257 (1986). https://doi.org/10.1007/BF00439395
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DOI: https://doi.org/10.1007/BF00439395