Correction of X-Linked Hyper-IgM Syndrome by Allogeneic Bone Marrow Transplantation

Abstract

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.1A variety of mutations of the gene encoding the CD40 ligand cause the immunodeficiency.2-6The 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. . . © 1995, Massachusetts Medical Society. All rights reserved.