Department of Immunohematology and Blood Transfusion, Kasturba Medical College Manipal, Manipal Academy of Higher Education, Manipal, Karnataka, India.
Department of Immunohematology and Blood Transfusion, Kasturba Medical College Manipal, Manipal Academy of Higher Education, Manipal, Karnataka, India.
Transfus Apher Sci. 2023 Apr;62(2):103583. doi: 10.1016/j.transci.2022.103583. Epub 2022 Oct 18.
Thromboembolic events are rare but one of the fatal complications in thalassemia. Assessment of the hypercoagulable state is not done regularly, and we have assessed the utility of Thromboelastography (TEG) for monitoring the activation of the coagulation pathway in patients with thalassemia.
A prospective single-center cohort study was conducted in a tertiary care set-up. Transfusion Dependent Thalassemia patients registered with the pediatric unit were screened for hypercoagulability using TEG during six months of the study period and followed up for three years for the development of thromboembolic events. Patient demographics, history of splenectomy, Serum ferritin levels and annual red cell transfusion requirement (mL/kg/year) were assessed. TEG parameters used were R time, K time, alpha angle, Maximum amplitude, Clot index, and Lysis 30. The thrombin generation test (V Curve) obtained from the first-degree derivate of the TEG velocity curve was also used for analysis.
A total of 34 patients were recruited during the six months study period with an average age of 10.6 years ( ± 5.47). The average pre-transfusion hemoglobin level and the volume of packed red cells received were 7.24 g/dL and 152.82 mL/kg/year respectively. The TEG tracing was suggestive of a hypercoagulable state in 58.82% of patients. The mean values of angle (70.74), MA (64.16), CI (2.65) and TG (774.43) in TDT patients compared to age matched reference range (62.81, 57.99, 0.8, 577.83 respectively) was suggestive of prothrombotic changes. Annual blood transfusion requirement was negatively correlated with hypercoagulable status (-0.344, CI= -0.68 to 0.08). One out of 34 patients developed corona radiata infarct (with annual blood requirement; 112.7 mL/kg/Year). The risk to develop a hypercoagulable state appeared to be higher when the volume of RBCs transfused was less than 154 mL/kg/Year.
TDT patients are at risk of developing thromboembolism, and screening with TEG may be useful to identify those at high risk.
血栓栓塞事件虽然罕见，但却是地中海贫血症的致命并发症之一。目前并未对高凝状态进行常规评估，本研究旨在评估血栓弹力图（TEG）在监测地中海贫血症患者凝血途径激活方面的作用。
这是一项在三级医疗中心进行的前瞻性单中心队列研究。在研究期间的 6 个月内，对登记在儿科病房的输血依赖型地中海贫血症患者使用 TEG 进行高凝状态筛查，并在 3 年内对血栓栓塞事件的发生情况进行随访。评估患者的人口统计学特征、脾切除术史、血清铁蛋白水平和每年红细胞输注需求（mL/kg/年）。使用的 TEG 参数包括 R 时间、K 时间、α角、最大振幅、凝血指数和 30 分钟溶解率。还使用 TEG 速度曲线的一阶导数获得的凝血酶生成试验（V 曲线）进行分析。
在 6 个月的研究期间，共招募了 34 名患者，平均年龄为 10.6 岁（±5.47）。平均输血前血红蛋白水平和接受的浓缩红细胞体积分别为 7.24g/dL 和 152.82mL/kg/年。58.82%的患者 TEG 描记提示存在高凝状态。与年龄匹配的参考范围相比，TDT 患者的平均角度（70.74）、MA（64.16）、CI（2.65）和 TG（774.43）值分别为（62.81，57.99，0.8，577.83），提示存在血栓前变化。每年的输血需求与高凝状态呈负相关（-0.344，CI=-0.68 至 0.08）。34 名患者中有 1 名发生放射冠梗死（年输血需求为 112.7mL/kg/年）。当输注的红细胞量小于 154mL/kg/年时，发生高凝状态的风险似乎更高。
TDT 患者有发生血栓栓塞的风险，使用 TEG 进行筛查可能有助于识别高危患者。
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