Abstract
There have been recent suggestions in the literature that in x-linked agammaglobulinemia, precursor B-cells may be present in the marrow and, further, that the peripheral blood lymphocytes (PBL) can be stimulated to produce IgM under appropriate conditions. Utilizing a recently described hemolytic plaque assay (Fauci and Pratt, PNAS 73:3676, 1976) we studied the PBL of 7 patients and marrow of 1 patient for ability to generate PFC following polyclonal stimulation with pokeweed mitogen in a wide dose range (1:20 - 1:1000 final dilution/culture) for 6 days. No PFC were generated in either blood or marrow whereas PBL from normal donors studied simultaneously gave from 107-426 plaques/million cultured cells. No PFC were generated in the PBL (or marrow in 1 patient) even after addition of irradiated normal PBL (1200 R) which regularly provided help to normal autologous or allogeneic cells. In 2 patients, PBL demonstrated suppressor activity; adequate helper activity was seen in the single marrow sample and in the PBL of 4/5 patients after irradiation. These observations confirm the findings of several other investigators that x-linked agammaglobulinemia is indeed a primary defect of the B-cell line in which suppressor cells may or may not be present. (Supported by grants CA-08748, CA-17404, CA-19267, AI-11843, Berman Estate and Judith Harris Selig Foundation funds)
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Pahwa, S., Pahwa, R., Smithwick, E. et al. 724 FAILURE TO GENERATE PLAQUE FORMING CELLS (PFC) IN X-LINKED AGAMAGLOBULINEMIA. Pediatr Res 12 (Suppl 4), 484 (1978). https://doi.org/10.1203/00006450-197804001-00729
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DOI: https://doi.org/10.1203/00006450-197804001-00729