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重型再生障碍性贫血的治疗:骨髓移植与免疫疗法的比较。

Treatment of severe aplastic anemia: comparison of bone marrow transplantation to immunotherapy.

作者信息

Tzeng C H, Chen P M, Chuang M W, Liu J H, Hsieh R K, Liu C J, Fan S, Chen K Y, Yung C H, Wang S Y

出版信息

Zhonghua Yi Xue Za Zhi (Taipei). 1989 Jan;43(1):21-8.

PMID:2670137
Abstract

Between February 1985 and May 1988, sixteen patients with severe aplastic anemia (SAA) post multitransfusion were treated with either allogeneic bone marrow transplantation (BMT) (9) or immunosuppression (7). The latter group was further divided into two subgroups: horse anti-human thymocyte-lymphocyte globulin (ATG-ALG) (2) and high dose methylprednisolone (HDMP) (5). There were 8 males and 8 females, age ranged from 10 to 35 years for the BMT group and 19 to 56 for the immunosuppression group. As of analysis, 9 patients in the BMT group had been followed from 1 to 31 months after transplant (median,24). Graft rejection was noted in 2, both had positive mixed lymphocyte culture (MLC) index. Seven had full recovery of hematopoiesis. Of these 7 survivals, none developed acute graft-versus-host disease (GVHD), whereas 2 had chronic GVHD which resolved completely after a 9-month treatment with azathioprine and prednisolone. Kaplan-Meier survival probability at 31 month was 75%. In the immunosuppressive therapy group 2, who received ATG-ALG both failed the treatment, died 7 and 29 months later, respectively. There were 3 responders in the HDMP subgroup, 1 complete, 2 partial. The 1-year survival probability for this group was 42.9% compared with 75% in the BMT group (p greater than 0.10). However, the hematologic reconstitution and Karnofsky performance were complete in all 7 transplant survivals vs one of 3 in the immunosuppression group (p less than 0.01). This experience supports that for patients with SAA under the age of 40, BMT is the treatment of choice if an HLA-identical MLC-nonreactive marrow donor is available, if not, immunosuppression is an alternative approach.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

1985年2月至1988年5月期间,16例多次输血后发生严重再生障碍性贫血(SAA)的患者接受了异基因骨髓移植(BMT)(9例)或免疫抑制治疗(7例)。后一组又进一步分为两个亚组:马抗人胸腺细胞淋巴细胞球蛋白(ATG-ALG)(2例)和大剂量甲基泼尼松龙(HDMP)(5例)。共有8名男性和8名女性,BMT组年龄为10至35岁,免疫抑制组为19至56岁。截至分析时,BMT组9例患者在移植后1至31个月接受了随访(中位数为24个月)。2例出现移植物排斥,两者混合淋巴细胞培养(MLC)指数均为阳性。7例造血功能完全恢复。在这7例存活者中,无一例发生急性移植物抗宿主病(GVHD),而2例发生慢性GVHD,在用硫唑嘌呤和泼尼松龙治疗9个月后完全缓解。31个月时的Kaplan-Meier生存概率为75%。在免疫抑制治疗组中,接受ATG-ALG治疗的2例均治疗失败,分别于7个月和29个月后死亡。HDMP亚组中有3例缓解者,1例完全缓解,2例部分缓解。该组1年生存概率为42.9%,而BMT组为75%(p>0.10)。然而,7例移植存活者的血液学重建和卡氏功能状态均完全恢复,而免疫抑制组3例中仅1例如此(p<0.01)。这一经验支持,对于40岁以下的SAA患者,如果有HLA匹配且MLC无反应的骨髓供者,BMT是首选治疗方法;如果没有,则免疫抑制是一种替代方法。(摘要截短于250词)

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