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First-line allogeneic hematopoietic stem cell transplantation of HLA-matched sibling donors compared with first-line ciclosporin and/or antithymocyte or antilymphocyte globulin for acquired severe aplastic anemia.与一线使用环孢素和/或抗胸腺细胞或抗淋巴细胞球蛋白治疗获得性重型再生障碍性贫血相比,HLA匹配的同胞供者进行一线异基因造血干细胞移植的情况。
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First-line matched related donor hematopoietic stem cell transplantation compared to immunosuppressive therapy in acquired severe aplastic anemia.一线匹配相关供者造血干细胞移植与获得性重型再生障碍性贫血的免疫抑制治疗比较。
PLoS One. 2011 Apr 25;6(4):e18572. doi: 10.1371/journal.pone.0018572.
6
Current concepts in the pathophysiology and treatment of aplastic anemia.再生障碍性贫血病理生理学与治疗的当前概念
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重型再生障碍性贫血的治疗:韩国单一机构中骨髓移植和免疫抑制治疗的结果

The treatment of severe aplastic anemia: outcomes of bone marrow transplantation and immunosuppressive therapy in a single institution of Korea.

作者信息

Kim Inho, Yoon Sung Soo, Park Seonyang, Kim Byoung Kook, Kim Noe Kyeong

机构信息

Department of Internal Medicine, Seoul National University, College of Medicine, Seoul, Korea.

出版信息

J Korean Med Sci. 2003 Jun;18(3):365-71. doi: 10.3346/jkms.2003.18.3.365.

DOI:10.3346/jkms.2003.18.3.365
PMID:12808323
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3055062/
Abstract

The present study represents an analysis of 96 patients with severe aplastic anemia (SAA) treated in Seoul National University Hospital, Seoul, Korea between 1990 and 1999. Twenty-two patients were treated by allogeneic bone marrow transplantation (BMT) from HLA identical sibling donors and 74 by immunosuppressive therapy (IS) with antithymocyte globulin (ATG) or antilymphocyte globulin (ALG). There was no statistical difference between the two treatment groups in age, sex, disease duration, and previous transfusion amount. In the BMT group, grade II-IV acute graft versus host disease (GVHD) developed in 10% and chronic GVHD occurred in 33% of patient. Only one patient died from complication of transplantation (veno-occlusive disease). Of 74 patients who received IS treatment, 45% achieved a complete or partial response. Twenty patients died among IS treatment group. Major causes of death were hemorrhage (40%) and infection (55%). In the BMT group, the 5-yr overall survival (OS) was 95% after a median follow-up of 42 months. In the IS group, the 5-yr OS was 70% after a median follow-up of 49 months (p=0.04). In conclusion, the long-term survival rates of SAA in Koreans receiving BMT or IS were excellent compared with the Western data. Further evaluation on the prognosis of aplastic anemia in Asians should be done.

摘要

本研究分析了1990年至1999年期间在韩国首尔国立大学医院接受治疗的96例重型再生障碍性贫血(SAA)患者。22例患者接受了来自HLA相同同胞供者的异基因骨髓移植(BMT),74例患者接受了抗胸腺细胞球蛋白(ATG)或抗淋巴细胞球蛋白(ALG)的免疫抑制治疗(IS)。两个治疗组在年龄、性别、病程和既往输血量方面无统计学差异。在BMT组中,10%的患者发生了II-IV级急性移植物抗宿主病(GVHD),33%的患者发生了慢性GVHD。仅1例患者死于移植并发症(静脉闭塞性疾病)。在接受IS治疗的74例患者中,45%获得了完全或部分缓解。IS治疗组中有20例患者死亡。主要死亡原因是出血(40%)和感染(55%)。在BMT组中,中位随访42个月后,5年总生存率(OS)为95%。在IS组中,中位随访49个月后,5年OS为70%(p=0.04)。总之,与西方数据相比,接受BMT或IS治疗的韩国SAA患者的长期生存率很高。应进一步评估亚洲人再生障碍性贫血的预后。