Optimizing outcomes of hematopoietic stem cell transplantation for severe combined immunodeficiency

被引:11
作者
Cuvelier, Geoff D. E. [2 ]
Schultz, Kirk R. [2 ]
Davis, Jeff [2 ]
Hirschfeld, Aaron F. [2 ]
Junker, Anne K. [2 ]
Tan, Rusung [1 ]
Turvey, Stuart E. [1 ,2 ]
机构
[1] BC Childrens Hosp & Child & Family, Div Infect & Immunol Dis, Res Inst, Pathol & Lab Med, Vancouver, BC V5Z 4H4, Canada
[2] Univ British Columbia, Dept Pediat, Federat Clin Immunol Soc Ctr Excellence, Vancouver, BC V6T 1W5, Canada
关键词
Primary immunodeficiency; Hematopoietic stem cell transplantation; Severe combined immunodeficiency; BONE-MARROW-TRANSPLANTATION; CORD BLOOD TRANSPLANTATION; TERM IMMUNE RECONSTITUTION; SINGLE-CENTER; MEGADOSE TRANSPLANTATION; EUROPEAN EXPERIENCE; IMPROVED SURVIVAL; PERIPHERAL-BLOOD; CHILDREN; DEFICIENCY;
D O I
10.1016/j.clim.2009.01.003
中图分类号
R392 [医学免疫学]; Q939.91 [免疫学];
学科分类号
100102 ;
摘要
This FOCIS Centers of Excelience Short Analytical Review is based on the clinical vignette of two boys from the same family with very different outcomes following hematopoietic stem cell transplantation (HSCT) for X-linked severe combined immunodeficiency (SCID). We review the kinetics of immune reconstitution following HSCT in SCID and emphasize the latest information regarding optimizing transplant outcomes for this disorder. The cases illustrate the difficulties and controversies surrounding the optimal strategies for planning SCID transplants. Specifically, we will focus on 3 areas of current debate and investigation: (i) factors involved in donor selection; (ii) the role of pretransplant conditioning; and (iii) benefits of early HSCT for SCID. (C) 2009 Elsevier Inc. All rights reserved.
引用
收藏
页码:179 / 188
页数:10
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