https://scholars.lib.ntu.edu.tw/handle/123456789/538672
標題: | Hematopoietic stem cell transplantation in Taiwanese children with primary immunodeficiency | 作者: | YUNG-LI YANG MENG-YAO LU SHIANN-TANG JOU KAI-HSIN LIN DONG-TSAMN LIN |
公開日期: | 2005 | 卷: | 104 | 期: | 2 | 起(迄)頁: | 101-106 | 來源出版物: | Journal of the Formosan Medical Association | 摘要: | Background and Purpose: Since 1968 it has been known that hematopoietic stem cell transplantation (HSCT) can ameliorate primary immunodeficiencies, but data on the long-term efficacy of this treatment in Taiwan are limited. This study analyzed the outcome of HSCT and the immune reconstruction in 10 children with primary immunodeficiencies in Taiwan. Methods: We retrospectively analyzed the outcome of HSCT in 10 children with primary immunodeficiencies between 1986 and 2002. The primary immunodeficiencies in these children included severe combined immunodeficiency (SCID) in 4, Wiskott-Aldrich syndrome (WAS) in 4, Chediak-Higashi syndrome (CHS) in 1, and leukocyte adhesion deficiency (LAD) in 1. The conditioning protocols included busulfan and cyclophosphamide in 2 patients with SCID, 3 patients with WAS, 1 patient with LAD and 1 patient with CHS. Anti-thymocyte globulin was given to only 1 patient with WAS and no conditioning therapy was given in 2 SCID patients. Graft-versus-host disease (GVHD) prophylaxis with cyclosporine (CsA) and methotrexate was prescribed in 6 children, CsA alone in 1, and CsA and T-cell depletion in 1. Results: Six patients were cured and 1 improved during a follow-up period from 3.5 years to 13 years after transplantation. Three patients died of severe sepsis. Three patients developed acute GVHD, which was grade 2 in 2 patients, and grade 3 in 1. Veno-occlusive disease developed in 1 patient and chronic GVHD with contracture of joints in 1. Conclusions: Our results support the benefits of allo-HSCT in children with primary immunodeficiencies. However, HSCT should be performed as soon as possible before severe infection develops. |
URI: | https://www.scopus.com/inward/record.uri?eid=2-s2.0-20144375960&partnerID=40&md5=4396b6acb8ecdcba6dbecfabec4b29d2 https://scholars.lib.ntu.edu.tw/handle/123456789/538672 |
ISSN: | 0929-6646 | SDG/關鍵字: | busulfan; cyclophosphamide; cyclosporin; ganciclovir; methotrexate; thymocyte antibody; article; Chediak Higashi syndrome; child; clinical article; combined immunodeficiency; controlled study; graft versus host reaction; hematopoietic stem cell; hematopoietic stem cell transplantation; human; immune deficiency; immune function test; joint contracture; leukocyte adhesion deficiency; T cell depletion; Taiwan; vein occlusion; virus pneumonia; Wiskott Aldrich syndrome; female; graft versus host reaction; hematopoietic stem cell transplantation; immune deficiency; immunosuppressive treatment; infant; male; methodology; retrospective study; Female; Graft vs Host Disease; Hematopoietic Stem Cell Transplantation; Humans; Immunologic Deficiency Syndromes; Infant; Male; Retrospective Studies; Taiwan; Transplantation Conditioning |
顯示於: | 醫學系 |
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