Allali Slimane, Peyrard Thierry, Amiranoff Denise, Cohen Jérémie F, Chalumeau Martin, Brousse Valentine, de Montalembert Mariane
Department of Paediatrics, Necker Hospital for Sick Children, Paris Descartes University, Paris, France.
Laboratory of Excellence GR-Ex, Paris, France.
Br J Haematol. 2017 May;177(4):641-647. doi: 10.1111/bjh.14609. Epub 2017 Apr 12.
Patients with sickle cell disease (SCD) show a high prevalence of red blood cell (RBC) alloimmunization, but few studies have focused on children. We aimed to study the prevalence and risk factors of RBC alloimmunization in SCD children. We retrospectively analysed the medical and transfusion files for 245 SCD children hospitalized in our centre in 2014 and included 175 patients who had received at least one RBC unit in their lifetime. The main clinical and immuno-haematological characteristics of alloimmunized and non-alloimmunized patients were compared. The prevalence of alloimmunization was 13·7% [95% confidence interval (CI) (8·6-18·6)], and 7·4% [95% CI (3·5-11·3)] after excluding the probable irregular natural antibodies (anti-M, anti-Le , anti-Le , anti-Le ). Main risk factors for alloimmunization were increased number of RBC units received (median of 65 vs. 10 units per patient; P = 0·01) and the presence of one or more red cell autoantibodies (46·2% vs. 4·7%; P < 0·0001). The alloimmunization rate was higher for episodically transfused than chronically transfused patients (1·43 vs. 0·24/100 units received; P < 0·001). The presence of red cell autoantibodies appears to be a major risk factor for alloimmunization in SCD children and could justify specific transfusion guidelines.
镰状细胞病(SCD)患者红细胞(RBC)同种免疫的患病率很高,但针对儿童的研究较少。我们旨在研究SCD儿童RBC同种免疫的患病率及危险因素。我们回顾性分析了2014年在我们中心住院的245例SCD儿童的病历和输血记录,纳入了175例一生中至少接受过1个RBC单位输血的患者。比较了同种免疫患者和非同种免疫患者的主要临床和免疫血液学特征。同种免疫的患病率为13.7%[95%置信区间(CI)(8.6 - 18.6)],排除可能的不规则天然抗体(抗-M、抗-Lea、抗-Leb、抗-Ley)后为7.4%[95%CI(3.5 - 11.3)]。同种免疫的主要危险因素是接受RBC单位数量增加(每位患者中位数为65个单位 vs. 10个单位;P = 0.01)以及存在一种或多种红细胞自身抗体(46.2% vs. 4.7%;P < 0.0001)。间歇性输血患者的同种免疫率高于长期输血患者(每接受100个单位分别为1.43 vs. 0.24;P < 0.001)。红细胞自身抗体的存在似乎是SCD儿童同种免疫的主要危险因素,这可能为制定特定的输血指南提供依据。