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Free access | 10.1172/JCI109473
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
Find articles by Geha, R. in: JCI | PubMed | Google Scholar
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
Find articles by Hyslop, N. in: JCI | PubMed | Google Scholar
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
Find articles by Alami, S. in: JCI | PubMed | Google Scholar
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
Find articles by Farah, F. in: JCI | PubMed | Google Scholar
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
Find articles by Schneeberger, E. in: JCI | PubMed | Google Scholar
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
Find articles by Rosen, F. in: JCI | PubMed | Google Scholar
Published August 1, 1979 - More info
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.
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