Abstract
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 with-out complications. Complete chimerism was demonstrated and post-transplant oxidative metabolism was normal. The patient is asymptomatic 30 months after the graft.
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Abbreviations
- BMT :
-
bone marrow transplantation
- CGD :
-
chronic granulomatous disease
References
Baehner RL, Nathan D (1968) Quantitative Nitroblue Tetrazolium test in chronic granulomatous disease. N Engl J Med 278:971–975
Bartolomeo P, Di Girolamo G, Angrilli F, Schettini F, De Mattia D, Manzionna MM, Dragani A, lacone A, Torlontano G (1968) Reconstitution of normal neutrophil function in chronic granulomatous disease by bone marrow transplantation. Bone Marrow Transplant 4:695–700
Fischer A, Segal AW, Seger R, Weening RS (1993) The management of chronic granulomatous disease. Eur J Pediatr 152:896–899
Gallin JI, Malech HL (1990) Update on chronic granulomatous diseases of childhood. JAMA 263:1533–1537
Gorlin JB, Stossel TP (1993) The phagocyte system: structure and function. In: Nathan DG, Oski FA (eds) Hematology of infancy and childhood. W. B. Saunders, Philadelphia, pp 882–903
Hobbs JR, Monteil M, McCluskey DR, Jurges E, Eltumi M (1992) Chronic granulomatous disease 100% corrected by displacement bone marrow transplantation from a volunteer unrelated donor. Eur J Pediatr 15:806–810
Kamani N, August CS, Douglas SD, Burkey E, Etzioni A, Lischner HW (1984) Bone marrow transplantation in chronic granulomatous disease. J Pediatr 105:42–46
Kamani N, August CS, Campbell DE, Hassan NF, Douglas SD (1988) Marrow transplantation in chronic granulomatous disease: an update, with 6-year follow-up. J Pediatr 113:697–700
Lehrer R, (1972) The fungicidal activity of human leukocytes. In: Williams and M. H. Fundenberg (eds) Phagocytic mechanisms in health and disease. Intercontinental Medical Book Co, New York, pp 151–165
Mouy R, Fischer A, Vilmer E, Seger R, Griscelli C (1989) Incidence, severity and prevention of infections in chronic granulomatous disease. J Pediatr 114:555–560
Rappeport JM, Newburger PE, Goldblum RM, Goldman AS, Nathan DG, Parkman R (1982) Allogenic bone marrow transplantation for chronic granulomatous disease. J Pediatr 101:952–955
Yang KD, Hill HR (1991) Neutrophil function disorders: Pathophysiology, prevention and therapy. J Pediatr 119:343–354
Zintl F, Herman J, Fuch D, Prager J, Muller A, Reiners B, Fuller J (1991) Correction of fatal genetic disease using bone marrow transplantation. Kinderarztl-Prax 59:10–15
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Calviño, M.C., Maldonado, M.S., Otheo, E. et al. Bone marrow transplantation in chronic granulomatous disease. Eur J Pediatr 155, 877–879 (1996). https://doi.org/10.1007/BF02282837
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DOI: https://doi.org/10.1007/BF02282837