Bone marrow transplantation in chronic granulomatous disease.

Abstract

UNLABELLED:

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.

CONCLUSION:

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.

• Authors: M C Calviño, M S Maldonado, E Otheo, A Muñoz, J M Couselo, C Burgaleta
• Journal: European journal of pediatrics (Eur J Pediatr) Vol. 155 Issue 10 Pg. 877-9 (Oct 1996) ISSN: 0340-6199 [Print] Germany
• PMID: 8891557 (Publication Type: Case Reports, Journal Article)

Chemical References

• Immunosuppressive Agents
• Cyclophosphamide
• Busulfan

Topics

• Bone Marrow Transplantation
• Busulfan (administration & dosage)
• Child, Preschool
• Cyclophosphamide (administration & dosage)
• Granulomatous Disease, Chronic (genetics, immunology, therapy)
• Humans
• Immunosuppressive Agents (administration & dosage)
• Male
• Tissue Donors