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[再生障碍性贫血的诊断与治疗]

[Diagnosis and treatment for aplastic anemia].

作者信息

Usuki Kensuke

机构信息

Department of Hematology, Medical Center Tokyo.

出版信息

Rinsho Ketsueki. 2021;62(8):922-930. doi: 10.11406/rinketsu.62.922.

Abstract

Treatments of aplastic anemia comprise supportive therapy and aplastic anemia-specific therapy to recover from hematopoiesis. Supportive therapy includes transfusion, granulocyte colony-stimulating factor, and iron chelation therapy in addition to symptomatic treatment. Aplastic anemia-specific treatments that aim to achieve hematopoietic recovery are immunosuppressive therapy, thrombopoietin receptor agonist (TPO-RA) treatment, allogeneic hematopoietic stem cell transplantation, and anabolic hormone therapy. Although the transplantation achieves complete recovery of hematopoiesis (healing), there is a risk of death from transplant-related complications. The most effective drug therapy is the combination of TPO-RA and the immunotherapy combined with anti-thymocyte globulin and cyclosporine. This treatment is also effective against secondary, drug-induced, or hepatitis-associated aplastic anemia. In the treatment of aplastic anemia, the treatment choice is made based on the disease severity and patient ages.

摘要

再生障碍性贫血的治疗包括支持性治疗和针对再生障碍性贫血的特异性治疗,以恢复造血功能。支持性治疗除对症治疗外,还包括输血、粒细胞集落刺激因子和铁螯合疗法。旨在实现造血恢复的再生障碍性贫血特异性治疗方法包括免疫抑制治疗、血小板生成素受体激动剂(TPO-RA)治疗、异基因造血干细胞移植和合成代谢激素治疗。尽管移植可实现造血功能的完全恢复(治愈),但存在因移植相关并发症而死亡的风险。最有效的药物治疗是TPO-RA与免疫疗法联合抗胸腺细胞球蛋白和环孢素。这种治疗方法对继发性、药物性或肝炎相关性再生障碍性贫血也有效。在再生障碍性贫血的治疗中,治疗方案的选择基于疾病严重程度和患者年龄。

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