Bone marrow transplantation in chronic granulomatous disease.
We present a 5-year-old boy with a severe form of X-linked chronic granulomatous disease and hypersensitivity to sulphamides preventing prophylaxis with trimethoprim-sulphomethoxazole. Bone marrow transplantation was performed after preconditioning with busulphan and cyclophosphamide. The immediate post-transplant period was without complications. Complete chimerism was demonstrated and post-transplant oxidative metabolism was normal. The patient is asymptomatic 30 months after the graft.


CONCLUSIONS
Bone marrow transplantation in cases of chronic granulomatous disease is controversial, although it could be useful in selected very severe cases in which prophylactic therapy is problematic.
Servicio de pediatría, Hospital Ramón y Cajal, Madrid, Spain.
M C
MC
Calviño
M S
MS
Maldonado
E
E
Otheo
A
A
Muñoz
J M
JM
Couselo
C
C
Burgaleta
D016026:Bone Marrow Transplantation; D002066:Busulfan / Q000008:administration & dosage; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000008:administration & dosage; D006105:Granulomatous Disease, Chronic / Q000235:genetics / Q000276:immunology / Q000628:therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D008297:Male; D014019:Tissue Donors
D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D002066:Busulfan