Bone marrow transplantation as treatment for X-linked immunodeficiency with hyper-IgM

Bone Marrow Transplant. 1998 Dec;22(11):1111-4. doi: 10.1038/sj.bmt.1701497.

Abstract

We report a 10-year-old boy with a severe form of immunodeficiency with hyper-IgM who underwent successful bone marrow transplantation with his HLA-matched sister as donor. Busulfan (20 mg/kg) and cyclophosphamide (200 mg/kg) were used as conditioning. The post-transplant course was uneventful. He is alive 25 months later with full hematological and immunological reconstitution.

Publication types

  • Case Reports

MeSH terms

  • B-Lymphocytes / immunology
  • Bone Marrow Transplantation*
  • CD40 Ligand
  • Child
  • Chimera / genetics
  • Female
  • Genetic Linkage
  • Humans
  • Hypergammaglobulinemia / genetics
  • Hypergammaglobulinemia / therapy*
  • Immunoglobulin M / blood*
  • Immunologic Deficiency Syndromes / genetics
  • Immunologic Deficiency Syndromes / therapy*
  • Male
  • Membrane Glycoproteins / deficiency
  • Membrane Glycoproteins / genetics
  • Point Mutation
  • T-Lymphocytes / immunology
  • Transplantation, Homologous
  • X Chromosome / genetics

Substances

  • Immunoglobulin M
  • Membrane Glycoproteins
  • CD40 Ligand