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Original Article
Brief Report
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Volume 333:426-429 August 17, 1995 Number 7
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Correction of X-Linked Hyper-IgM Syndrome by Allogeneic Bone Marrow Transplantation
Caroline Thomas, M.D., Geneviève de Saint Basile, M.D., Ph.D., Françoise Le Deist, M.D., Ph.D., Didier Theophile, M.D., Malika Benkerrou, M.D., Elie Haddad, M.D., Stéphane Blanche, M.D., and Alain Fischer, M.D., Ph.D.

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The X-linked hyper-IgM syndrome is a rare immunodeficiency disease in which the ability of B cells to switch immunoglobulin production from IgM to IgG, IgA, and IgE is defective.1 A variety of mutations of the gene encoding the CD40 ligand cause the immunodeficiency.2,3,4,5,6 The functional effect of the mutation is that the CD40 ligand on T cells cannot interact with the CD40 glycoprotein on the surface of B cells. This interaction normally mediates immunoglobulin class switching by B cells. The deficiency of IgG and IgA leads to recurrent infections of the respiratory tract that can be prevented by intravenous immune . . . [Full Text of this Article]

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From the Unité d'Immunohématologie (C.T., M.B., E.H., S.B., A.F.), INSERM Unité 429 (G.S.B., F.L.D., A.F.), and the Laboratoire de Cytogénétique, Hôpital des Enfants-Malades (D.T.) — all in Paris.

Address reprint requests to Dr. Fischer at INSERM Unité 429, Hôpital Necker, 149 Rue de Sevres, 75015 Paris, France.

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