依赖输血的地中海贫血症中的红细胞同种免疫：系统评价。
Red blood cell alloimmunisation in transfusion-dependent thalassaemia: a systematic review.
机构信息
Italian National Blood Centre, National Institute of Health, Rome, Italy.
Department of Haematology and Transfusion Medicine, "Carlo Poma" Hospital, Mantua, Italy.
出版信息
Blood Transfus. 2019 Jan;17(1):4-15. doi: 10.2450/2019.0229-18.
BACKGROUND
Chronic red blood cell transfusion is the first-line treatment for severe forms of thalassaemia. This therapy is, however, hampered by a number of adverse effects, including red blood cell alloimmunisation. The aim of this systematic review was to collect the current literature data on erythrocyte alloimmunisation.
MATERIALS AND METHODS
We performed a systematic search of the literature which identified 41 cohort studies involving 9,256 patients.
RESULTS
The prevalence of erythrocyte alloimmunisation was 11.4% (95% CI: 9.3-13.9%) with a higher rate of alloimmunisation against antigens of the Rh (52.4%) and Kell (25.6%) systems. Overall, alloantibodies against antigens belonging to the Rh and Kell systems accounted for 78% of the cases. A higher prevalence of red blood cell alloimmunisation was found in patients with thalassaemia intermedia compared to that among patients with thalassaemia major (15.5 vs 12.8%).
DISCUSSION
Matching transfusion-dependent thalassaemia patients and red blood cell units for Rh and Kell antigens should be able to reduce the risk of red blood cell alloimmunisation by about 80%.
背景
慢性红细胞输注是治疗重型地中海贫血的一线治疗方法。然而，这种治疗方法受到许多不良反应的阻碍，包括红细胞同种免疫。本系统评价的目的是收集红细胞同种免疫的当前文献数据。
材料和方法
我们进行了系统的文献检索，共纳入了 41 项队列研究，涉及 9256 名患者。
结果
红细胞同种免疫的患病率为 11.4%（95%CI：9.3-13.9%），对 Rh（52.4%）和 Kell（25.6%）系统抗原的同种免疫率更高。总体而言，针对 Rh 和 Kell 系统抗原的同种抗体占病例的 78%。中间型地中海贫血患者的红细胞同种免疫发生率高于重型地中海贫血患者（15.5%比 12.8%）。
讨论
为 Rh 和 Kell 抗原匹配依赖输血的地中海贫血患者和红细胞单位，应该能够将红细胞同种免疫的风险降低约 80%。
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