促红细胞生成素刺激剂在治疗抵抗性自身免疫性溶血性贫血患者中的作用。
The effect of erythropoiesis-stimulating agents in patients with therapy-refractory autoimmune hemolytic anemia.
机构信息
Institute of Transfusion Medicine, Charité-Universitätsmedizin - Campus Virchow-Klinikum, Berlin, Germany.
Department for Internal Medicine / Hematology and Oncology. Evangelisches Krankenhaus Wesel, Wesel, Germany.
出版信息
Transfus Med Hemother. 2014 Nov;41(6):462-8. doi: 10.1159/000366244. Epub 2014 Sep 15.
BACKGROUND
Many patients with autoimmune hemolytic anemia (AIHA) do not respond to standard therapy and/or may develop severe complications which can be of fatal outcome. There is some evidence that erythropoiesis-stimulating agents (ESAs) may be helpful in the management of such patients.
METHODS
We describe the effect of ESAs in 12 new patients with therapy-refractory AIHA (7 of warm type and 5 of cold type) and review 5 previously reported cases. Serological testing was performed using standard methods.
RESULTS
All patients responded well to treatment with ESAs. At least 5 of the 17 patients demonstrated complete recovery, and none of the patients developed significant adverse reactions due to treatment with ESAs.
CONCLUSION
The mechanism by which ESAs improves hemolysis in AIHA is not completely clear. In addition to increased production and prolonged RBC survival, it may inhibit eryptosis (programmed cell death). ESAs represent a new option in the treatment of decompensated and/or refractory AIHA of warm and cold type. However, more information is required to assess which patients can be treated with ESAs.
背景
许多自身免疫性溶血性贫血（AIHA）患者对标准治疗无反应和/或可能发生严重并发症，这些并发症可能导致致命后果。有一些证据表明，红细胞生成刺激剂（ESA）可能有助于此类患者的治疗。
方法
我们描述了 ESA 对 12 例治疗抵抗的 AIHA 新患者（7 例温抗体型和 5 例冷抗体型）的治疗效果，并回顾了 5 例以前报道的病例。使用标准方法进行血清学检测。
结果
所有患者对 ESA 治疗反应良好。至少有 5 例患者完全缓解，且没有患者因 ESA 治疗而发生明显不良反应。
结论
ESA 改善 AIHA 溶血的机制尚不完全清楚。除了增加红细胞生成和延长 RBC 存活时间外，ESA 还可能抑制红细胞凋亡（程序性细胞死亡）。ESA 代表了温型和冷型失代偿和/或难治性 AIHA 治疗的新选择。然而，需要更多信息来评估哪些患者可以用 ESA 治疗。
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