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J Clin Invest. 1979 August; 64(2): 385–391. doi: 10.1172/JCI109473. | PMCID: PMC372130 |
Hyper Immunoglobulin M Immunodeficiency (Dysgammaglobulinemia) PRESENCE OF IMMUNOGLOBULIN M-SECRETING PLASMACYTOID CELLS IN PERIPHERAL BLOOD AND FAILURE OF IMMUNOGLOBULIN M-IMMUNOGLOBULIN G SWITCH IN B-CELL DIFFERENTIATION Raif S. Geha, Newton Hyslop, Samih Alami, Fuad Farah, Eveline E. Schneeberger, and Fred S. Rosen Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02114 Department of Medicine, Harvard Medical School, Boston, Massachusetts 02114 Department of Pathology, Harvard Medical School, Boston, Massachusetts 02114 Division of Allergy and Immunology, Department of Medicine, Children's Hospital Medical Center, Boston, Massachusetts 02114 Department of Pathology, Children's Hospital Medical Center, Boston, Massachusetts 02114 Infectious Disease Unit, Department of Medicine, Massachusetts General Hospital, Boston, Massachusetts 02114 Department of Medicine, American University Medical Center, Beirut, Lebanon Department of Pediatrics, American University Medical Center, Beirut, Lebanon Abstract The peripheral blood lymphocytes of nine patients with hyper immunoglobulin (Ig)M immunodeficiency were studied in an attempt to define the cellular basis of this disorder. B cells were normal in number but qualitatively abnormal in all patients. Approximately one-half of the B cell consisted of small lymphocytes (7-9 μm in diameter) bearing surface IgM and IgD, as well as C3 receptors. These cells were driven to secrete IgM but not IgG after in vitro stimulation by pokeweed mitogen. In the blood there were also large lymphocytes (10-14 μm in diameter) that possessed surface as well as intracytoplasmic IgM but lacked C3 receptors. These cells spontaneously secreted large amounts of IgM in vitro and on electron microscopy were found to be rich in rough endoplasmic reticulum. Such a subpopulation of lymphoid cells was not detected in normal peripheral blood and was unique for all patients with hyper IgM immunodeficiency studied. T cells from all patients were normal in number and in function both in vivo and in vitro and were able to generate adequate T-cell help to support IgG synthesis by normal B cells. No evidence was obtained for T cells capable of suppressing normal IgG synthesis in any of the patients after coculture with normal peripheral blood lymphocytes. The defect in hyper IgM immunodeficiency is intrinsic to B cells, which fail to switch from IgM to IgG synthesis. Full text Full text is available as a scanned copy of the original print version. Get a printable copy (PDF file) of the complete article (1.2M), or click on a page image below to browse page by page. Links to PubMed are also available for Selected References. Images in this article Click on the image to see a larger version. These references are in PubMed. This may not be the complete list of references from this article. - Rosen FS, Janeway CA. The gamma globulins. 3. The antibody deficiency syndromes. N Engl J Med. 1966 Sep 29;275(13):709–contd. [PubMed]
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