Characterization of B cells in severe combined immunodeficiency disease Journal Article
| Authors: | Small, T. N.; Keever, C.; Collins, N.; Dupont, B.; O'Reilly, R. J.; Flomenberg, N. |
| Article Title: | Characterization of B cells in severe combined immunodeficiency disease |
| Abstract: | The circulating lymphoid cells of eight consecutive untreated infants with severe combined immunodeficiency disease (SCID) with B cells were analyzed for surface marker expression and function. The B cells of these children expressed sIg, HLA-DR, CD19 (B4), CD20 (B1), CD21 (B2), Leu-8, and lacked expression of CD10 (CALLA), as do normal peripheral blood B lymphocytes. SCID B cells also expressed antigens that are normally absent or present on only a minor subset of circulating adult B lymphocytes, including CD1c (M241), CD38 (OKT10), CD23 (PL13), with or without concomitant CD5 (Leu-1) expression. The B cells of these children were capable of proliferating in vitro when stimulated with Staphylococcus aureus Cowan I. However, in the presence of pokeweed mitogen, S. aureus Cowan I, and normal T cells, the sIg+ cells of these children produced only IgM. Studies performed on normal B cells obtained from cord blood, young children, and adults reveal that whereas cord blood B cells are predominantly CD1c, CD38, and CD23 positive, B-cell expression of these antigens decreases with age. Cord blood B cells, similar to SCID B cells, produce only IgM when stimulated in vitro with pokeweed mitogen and S. aureus Cowan I. Based on these observations, we hypothesize that SCID B cells represent a population of B cells present during normal B-cell ontogeny which becomes a minor subset when an individual develops full immunologic competence. © 1989. |
| Keywords: | child; clinical article; comparative study; flow cytometry; lymphocyte proliferation; heredity; b lymphocyte; b-lymphocytes; fluorescent antibody technique; antibodies, monoclonal; cell culture; infant; infant, newborn; cell line, transformed; enzyme-linked immunosorbent assay; monocytes; cell separation; cytochemistry; combined immunodeficiency; immunologic deficiency syndromes; antigens, surface; lymphocyte surface marker; human; male; female; priority journal; support, non-u.s. gov't; support, u.s. gov't, p.h.s. |
| Journal Title: | Human Immunology |
| Volume: | 25 |
| Issue: | 3 |
| ISSN: | 0198-8859 |
| Publisher: | Elsevier Inc. |
| Date Published: | 1989-07-01 |
| Start Page: | 181 |
| End Page: | 193 |
| Language: | English |
| DOI: | 10.1016/0198-8859(89)90081-5 |
| PUBMED: | 2670851 |
| PROVIDER: | scopus |
| DOI/URL: | |
| Notes: | Article -- Source: Scopus |
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