Bone marrow transplantation as treatment for X-linked immunodeficiency with hyper-IgM.
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.
Bordigoni
P
P
Bone Marrow Transplantation Unit, Children's Hospital, Nancy, France.
Auburtin
B
B
Carret
A S
AS
Schuhmacher
A
A
Humbert
J C
JC
Le Deist
F
F
Sommelet
D
D
D001402:B-Lymphocytes / Q000276:immunology; D016026:Bone Marrow Transplantation; D023201:CD40 Ligand; D002648:Child; D002678:Chimera / Q000235:genetics; D005260:Female; D008040:Genetic Linkage; D006801:Humans; D006942:Hypergammaglobulinemia / Q000235:genetics / Q000628:therapy*; D007075:Immunoglobulin M / Q000097:blood*; D007153:Immunologic Deficiency Syndromes / Q000235:genetics / Q000628:therapy*; D008297:Male; D008562:Membrane Glycoproteins / Q000172:deficiency / Q000235:genetics; D017354:Point Mutation; D013601:T-Lymphocytes / Q000276:immunology; D014184:Transplantation, Homologous; D014960:X Chromosome / Q000235:genetics
D007075:Immunoglobulin M; D008562:Membrane Glycoproteins; D023201:CD40 Ligand