Correction of purine nucleoside phosphorylase deficiency by transplantation of allogeneic bone marrow from a sibling

被引:21
作者
Broome, CB
Graham, ML
Saulsbury, FT
Hershfield, MS
Buckley, RH
机构
[1] DUKE UNIV, MED CTR, DEPT PEDIAT, DURHAM, NC 27710 USA
[2] DUKE UNIV, MED CTR, DEPT IMMUNOL, DURHAM, NC 27710 USA
[3] DUKE UNIV, MED CTR, DEPT INTERNAL MED, DURHAM, NC 27710 USA
[4] UNIV VIRGINIA, SCH MED, DEPT PEDIAT, CHARLOTTESVILLE, VA 22908 USA
关键词
D O I
10.1016/S0022-3476(96)70285-8
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
Deficiency of the purine salvage pathway enzyme purine nucleoside phosphorylase causes a combined immunodeficiency and neurologic abnormalities and is usually fatal in childhood, We report the first successful transplantation of bone marrow from a sibling with identical class II human leukocyte antigens in this condition, demonstrating correction of both lymphocyte metabolic and functional abnormalities.
引用
收藏
页码:373 / 376
页数:4
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