Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, 270 Rama VI Road, Ratchathewi District, Bangkok, 10400, Thailand.
Department of Nursing, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Ann Hematol. 2019 Jul;98(7):1593-1602. doi: 10.1007/s00277-019-03672-4. Epub 2019 Apr 5.
One complication of thalassemia is thromboembolism (TE), which is caused by an abnormal red blood cell surface, as well as endothelial and platelet activation. These findings are commonly observed in severe β-thalassemia. However, limited information on α-thalassemia exists. This study enrolled subjects with deletional and non-deletional α-thalassemia and normal controls (NC). Plasma and serum of subjects were tested for endothelial activation markers including thrombomodulin (TM), vascular cell adhesion molecule-1 (VCAM-1), and von Willebrand factor antigen as well as platelet activation markers including thromboxane B2 and platelet factor 4. A total of 179 subjects were enrolled: 29 in the deletional group (mean age 13.3 ± 4.4 years), 31 in the non-deletional group (mean age 12.9 ± 4.8 years), and 119 in the NC group (mean age 13.6 ± 3.0 years). Twenty nine percent of subjects in the non-deletional group received regular red blood cell transfusion and iron chelator administration. Serum ferritin level was higher in the non-deletional group than that in the deletional group. Multivariate analysis demonstrated that VCAM-1 and TM levels were increased significantly in α-thalassemia compared with NC group (816.8 ± 131.0 vs 593.9 ± 49.0 ng/ml, and 4.9 ± 0.7 vs 4.0 ± 0.4 ng/ml, P < 0.001 respectively). VCAM-1 and TM levels in the non-deletional group were significantly higher than that in the deletional group. The present study demonstrated endothelial activation in children with α-thalassemia disease, especially those in the non-deletional group, which might be one risk factor for TE in α-thalassemia disease.
地中海贫血症的一种并发症是血栓栓塞症（TE），其由异常的红细胞表面以及内皮细胞和血小板的激活引起。这些发现通常在严重的β地中海贫血症中观察到。然而，关于α地中海贫血症的信息有限。本研究纳入了缺失型和非缺失型α地中海贫血症患者以及正常对照组（NC）。对受试者的血浆和血清进行了内皮细胞激活标志物的检测，包括血栓调节蛋白（TM）、血管细胞黏附分子-1（VCAM-1）和血管性血友病因子抗原，以及血小板激活标志物，包括血栓烷 B2 和血小板因子 4。共纳入 179 名受试者：缺失组 29 名（平均年龄 13.3±4.4 岁），非缺失组 31 名（平均年龄 12.9±4.8 岁），NC 组 119 名（平均年龄 13.6±3.0 岁）。非缺失组中有 29%的受试者接受了定期红细胞输血和铁螯合剂治疗。非缺失组的血清铁蛋白水平高于缺失组。多变量分析表明，与 NC 组相比，α-地中海贫血症患者的 VCAM-1 和 TM 水平显著升高（816.8±131.0 vs 593.9±49.0 ng/ml 和 4.9±0.7 vs 4.0±0.4 ng/ml，P<0.001）。非缺失组的 VCAM-1 和 TM 水平明显高于缺失组。本研究表明，α-地中海贫血症患儿存在内皮细胞激活，尤其是非缺失型患儿，这可能是α-地中海贫血症发生 TE 的一个危险因素。
