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

被引：25

作者：

Aytekin, Caner ^{[1
]}

Yuksek, Mutlu ^{[1
]}

Dogu, Figen ^{[1
]}

Yagmurlu, Aydin ^{[2
]}

Yildiran, Alisan ^{[1
]}

Fitoz, Suat ^{[3
]}

Kologlu, Meltem ^{[2
]}

Babacan, Emel ^{[1
]}

Hershfield, Michael S. ^{[4
]}

Ikinciogullari, Aydan ^{[1
]}

机构：

[1] Ankara Univ, Sch Med, Dept Pediat Immunol & Allergy, TR-06100 Ankara, Turkey

[2] Ankara Univ, Sch Med, Dept Pediat Surg, TR-06100 Ankara, Turkey

[3] Ankara Univ, Sch Med, Dept Radiol, TR-06100 Ankara, Turkey

[4] Duke Univ, Med Ctr, Dept Med, Durham, NC 27710 USA

来源：

PEDIATRIC TRANSPLANTATION | 2008年 / 12卷 / 04期

关键词：

purine nucleoside phosphorylase deficiency; bone marrow transplantation; disseminated BCG infection;

D O I：

10.1111/j.1399-3046.2007.00890.x

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

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.

引用

页码：479 / 482

页数：4

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