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An unconditioned bone marrow transplantation in a child with purine nucleoside phosphorylase deficiency and its unique complication.

Authors :
Aytekin C
Yuksek M
Dogu F
Yagmurlu A
Yildiran A
Fitoz S
Kologlu M
Babacan E
Hershfield MS
Ikinciogullari A
Source :
Pediatric transplantation [Pediatr Transplant] 2008 Jun; Vol. 12 (4), pp. 479-82. Date of Electronic Publication: 2008 Jan 14.
Publication Year :
2008

Abstract

Purine nucleoside phosphorylase deficiency is a rare immunodeficiency syndrome characterized by recurrent infections, neurological dysfunction, and autoimmunity. Early diagnosis and hematopoietic stem cell transplantation may reverse the dismal prognosis in PNP deficiency. This report presents a new PNP deficiency case successfully transplanted without a conditioning regimen from an HLA-identical family donor, who developed a complication of disseminated BCG infection.

Details

Language :
English
ISSN :
1399-3046
Volume :
12
Issue :
4
Database :
MEDLINE
Journal :
Pediatric transplantation
Publication Type :
Academic Journal
Accession number :
18208442
Full Text :
https://doi.org/10.1111/j.1399-3046.2007.00890.x