Chronic granulomatous disease 100% corrected by displacement bone marrow transplantation from a volunteer unrelated donor

J. R. Hobbs, Michele Anne Monteil, D. R. McCluskey, E. Jurges, M. El Tumi

Research output: Contribution to journalArticle

34 Citations (Scopus)

Abstract

A boy whose chronic granulomatous disease (CGD) manifested in infancy, and whose elder brother had died at 7 years of age, had phagocytes with complete lack of functional cytochrome B-245 and which could not be induced by interferon gamma to achieve adequate staphylococcal killing. He underwent an elective displacement bone marrow transplant from a volunteer unrelated donor at the age of 8 months. This has achieved 100% replacement of the CGD granulocytes by those of the normal volunteer and the boy has since had a normal childhood for 3 years. Six previous transplants for CGD are briefly reviewed and illustrate that the host abnormal marrow must be completely displaced using an adequate dose of busulphan to ensure 100% stable engraftment of the donor's marrow and that this is best done under elective conditions before septic foci and irreversible organ damage have occurred. Criteria need to be developed to identify early those patients likely to have severe morbidity.

Original languageEnglish (US)
Pages (from-to)806-810
Number of pages5
JournalEuropean Journal of Pediatrics
Volume151
Issue number11
DOIs
StatePublished - Nov 1 1992
Externally publishedYes

Fingerprint

Chronic Granulomatous Disease
Unrelated Donors
Bone Marrow Transplantation
Volunteers
Bone Marrow
Transplants
Busulfan
Cytochromes
Phagocytes
Granulocytes
Interferon-gamma
Siblings
Healthy Volunteers
Tissue Donors
Morbidity

Keywords

  • Bone marrow transplantation
  • Chronic granulomatous disease
  • Matched unrelated donors

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Chronic granulomatous disease 100% corrected by displacement bone marrow transplantation from a volunteer unrelated donor. / Hobbs, J. R.; Monteil, Michele Anne; McCluskey, D. R.; Jurges, E.; El Tumi, M.

In: European Journal of Pediatrics, Vol. 151, No. 11, 01.11.1992, p. 806-810.

Research output: Contribution to journalArticle

Hobbs, J. R. ; Monteil, Michele Anne ; McCluskey, D. R. ; Jurges, E. ; El Tumi, M. / Chronic granulomatous disease 100% corrected by displacement bone marrow transplantation from a volunteer unrelated donor. In: European Journal of Pediatrics. 1992 ; Vol. 151, No. 11. pp. 806-810.
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