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.
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: P Di Bartolomeo; G Di Girolamo; F Angrilli; F Schettini; D De Mattia; M M Manzionna; A Dragani; A Iacone; G Torlontano Journal: Bone Marrow Transplant Date: 1989-11 Impact factor: 5.483
Authors: H L Malech; P B Maples; N Whiting-Theobald; G F Linton; S Sekhsaria; S J Vowells; F Li; J A Miller; E DeCarlo; S M Holland; S F Leitman; C S Carter; R E Butz; E J Read; T A Fleisher; R D Schneiderman; D E Van Epps; S K Spratt; C A Maack; J A Rokovich; L K Cohen; J I Gallin Journal: Proc Natl Acad Sci U S A Date: 1997-10-28 Impact factor: 11.205