title: Immunological reconstitution by allogeneic bone marrow transplantation in a child with the X-linked hyper-IgM syndrome.
abstract: A successful transplantation of sibling marrow in a patient with the X-linked hyper-IgM syndrome is reported. Engraftment of HLA-identical marrow cells was obtained, although complicated by grade I acute graft-versus-host disease. Expression of the CD40 ligand (CD40L, CD154) by activated T-cells from the recipient remained at low levels until 10 months after the transplantation, but then normalized. The patient is now fully competent in immune function without any episodes of severe infection 24 months later.
  
  
  CONCLUSIONS
  Allogeneic bone marrow transplantation is a reasonable therapeutic option for X-linked hyper-IgM syndrome if HLA-matched family donors are available. Whether dysregulation of CD40L expression causes post-transplant immunological abnormalities remains to be clarified.
authors.affiliation: Department of Paediatric Oncology, Institute of Development, Aging and Cancer, Tohoku University, Sendai, Japan.
authors.forename: S
authors.initials: S
authors.lastname: Kawai
authors.forename: Y
authors.initials: Y
authors.lastname: Sasahara
authors.forename: M
authors.initials: M
authors.lastname: Minegishi
authors.forename: S
authors.initials: S
authors.lastname: Tsuchiya
authors.forename: H
authors.initials: H
authors.lastname: Fujie
authors.forename: Y
authors.initials: Y
authors.lastname: Ohashi
authors.forename: S
authors.initials: S
authors.lastname: Kumaki
authors.forename: T
authors.initials: T
authors.lastname: Konno
mesh_terms: D000945:Antigens, Differentiation, T-Lymphocyte / Q000276:immunology; D016026:Bone Marrow Transplantation / Q000276:immunology; D019013:CD40 Antigens / Q000276:immunology; D023201:CD40 Ligand; D002675:Child, Preschool; D008040:Genetic Linkage; D006801:Humans; D006942:Hypergammaglobulinemia / Q000235:genetics / Q000276:immunology / Q000628:therapy*; D007075:Immunoglobulin M / Q000276:immunology; D008024:Ligands; D008297:Male; D008562:Membrane Glycoproteins / Q000276:immunology; D013577:Syndrome; D014184:Transplantation, Homologous; D014960:X Chromosome
chemical_list: D000945:Antigens, Differentiation, T-Lymphocyte; D019013:CD40 Antigens; D007075:Immunoglobulin M; D008024:Ligands; D008562:Membrane Glycoproteins; D023201:CD40 Ligand