Faculty of Medicine, University of Tunis El Manar, Tunis, Tunisia; Pediatric Immuno-Hematology Unit, Bone Marrow Transplantation Center Tunis, Tunis, Tunisia.
Faculty of Medicine, University of Tunis El Manar, Tunis, Tunisia; Pediatric Immuno-Hematology Unit, Bone Marrow Transplantation Center Tunis, Tunis, Tunisia.
Blood Cells Mol Dis. 2019 Nov;79:102342. doi: 10.1016/j.bcmd.2019.102342. Epub 2019 Jun 29.
In beta-thalassemia patients, erythrocyte autoantibodies can remain silent or lead to Autoimmune Hemolytic Anemia (AIHA).The aim of this study was to identify predictors of AIHA in beta-thalassemia patients with positive Direct Antiglobulin Test (DAT), in Tunisia. This longitudinal prognosis study was carried out on beta-thalassemia patients with a positive confirmed DAT. Predictors of AIHA were identified the Kaplan-Meier method. A Cox model analysis was used to identify independent predictors. Among 385 beta thalassemia patients, 87 developed positive DAT (22.6%). Autoimmune hemolytic anemia was occurred in 25 patients. Multivariate analysis showed that AIHA was independently associated with beta-thalassemia intermedia and similar family history of AIHA. Splenectomy in patients with positive DAT was independently associated with an increased risk of AIHA (HR = 6.175, CI: 2.049-18.612, p < 0.001). The risk of developing AIHA was higher during the first 72 transfusions. Autoimmune hemolytic anemia was significantly associated with polyspecific DAT (anti-complement and anti-IgG), blood group AB and prior alloimmunization. Whereas transfusion by phenotypic and leukoreduced blood was a protective factor. In summary, splenectomy after autoimmunization, prior alloimmunization, DAT specificity (IgG with complement), thalassemia intermedia, AB blood group and family history of AIHA were strongly associated with AIHA. Leukoreduced blood transfusion had a proven preventive role.
在β地中海贫血患者中，红细胞自身抗体可能处于静止状态，也可能导致自身免疫性溶血性贫血（AIHA）。本研究旨在鉴定突尼斯具有阳性直接抗球蛋白试验（DAT）的β地中海贫血患者发生 AIHA 的预测因子。这是一项前瞻性预后研究，纳入了 DAT 阳性的β地中海贫血患者。使用 Kaplan-Meier 法确定 AIHA 的预测因子。使用 Cox 模型分析确定独立预测因子。在 385 例β地中海贫血患者中，有 87 例 DAT 阳性（22.6%）。有 25 例发生自身免疫性溶血性贫血。多变量分析显示，β地中海贫血中间型和类似的 AIHA 家族史与 AIHA 独立相关。DAT 阳性患者行脾切除术与 AIHA 风险增加独立相关（HR=6.175，95%CI：2.049-18.612，p<0.001）。在首次输血后 72 小时内发生 AIHA 的风险更高。AIHA 与多特异性 DAT（抗补体和抗 IgG）、AB 血型和既往同种免疫显著相关。而输血时使用表型和白细胞减少血液是保护因素。总之，自身免疫后行脾切除术、既往同种免疫、DAT 特异性（IgG 结合补体）、β地中海贫血中间型、AB 血型和 AIHA 家族史与 AIHA 强烈相关。白细胞减少血液输注具有明确的预防作用。
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