突尼斯依赖输血的地中海贫血患者中自身免疫的高发生率。
High frequency of autoimmunization among transfusion-dependent Tunisian thalassaemia patients.
作者信息
Guirat-Dhouib N, Mezri M, Hmida H, Mellouli F, Kaabi H, Ouderni M, Zouari B, Hmida S, Bejaoui M
机构信息
Service d'immuno-hématologie pédiatrique, Centre National de Greffe de moelle osseuse 2, rue Djebel Lakhdar, Bab Saadoun 1006, Tunis, Tunisie.
出版信息
Transfus Apher Sci. 2011 Oct;45(2):199-202. doi: 10.1016/j.transci.2011.08.003. Epub 2011 Sep 1.
BACKGROUND
Limited data are available on the frequency of RBC alloimmunization and autoimmunization in transfusion-dependent Tunisian β thalassaemia patients.
MATERIALS AND METHODS
We analyzed the clinical and transfusion records of 130 patients (57 females and 73 males; mean age 119 months; range 12-11 months) with β thalassaemia major and who had regular blood transfusions for periods ranging from 12 to 311 months.
RESULTS
Of the 130 patients, ten (7.7%) developed RBC alloantibodies. The most common alloantibodies were directed against antigens in the Rh systems. Erythrocyte-autoantibodies as determined by a positive direct antiglobulin Coombs test, developed in 52(40%) patients with and without underlying RBC alloantibodies, thereby causing autoimmune haemolytic anaemia in eleven patients (21%).
CONCLUSIONS
Autoimmunization to erythrocyte antigens is a frequent complication in patients with β thalassaemia major. Several factors might have contributed to the high autoimmunization rate observed in this study, including non phenotypic blood exposure and alloantibody formation prior to positive Coombs test.
背景
关于依赖输血的突尼斯β地中海贫血患者红细胞同种免疫和自身免疫发生频率的数据有限。
材料与方法
我们分析了130例重型β地中海贫血患者（57例女性和73例男性；平均年龄119个月；范围12 - 311个月）的临床和输血记录，这些患者定期输血12至311个月。
结果
130例患者中，10例（7.7%）产生了红细胞同种抗体。最常见的同种抗体针对Rh系统中的抗原。通过直接抗球蛋白库姆斯试验阳性确定的红细胞自身抗体，在52例（40%）有或无潜在红细胞同种抗体的患者中出现，从而导致11例患者（21%）发生自身免疫性溶血性贫血。
结论
红细胞抗原自身免疫是重型β地中海贫血患者常见的并发症。本研究中观察到的高自身免疫率可能由多种因素导致，包括非表型血液暴露以及在库姆斯试验阳性之前形成的同种抗体。
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