Department of Medical Laboratory Sciences, Faculty of Allied Health Sciences, Health Sciences Center, Kuwait University, Kuwait City, Kuwait.
Int J Lab Hematol. 2023 Dec;45(6):945-952. doi: 10.1111/ijlh.14135. Epub 2023 Jul 12.
INTRODUCTION: Alterations in the endothelium and endothelial adhesion proteins such as von Willebrand factor (vWF) play major roles in hypercoagulability in thalassemia. vWF protein release leads to platelet aggregation and thrombi formation at the site of vascular injury. It is then degraded by the proteolytic enzyme ADAMTS13. Thrombospondin-1 is a multifactorial glycoprotein, which was reported to compete with ADAMTS13 for sites of vWF proteolysis. In this study, levels of vWF, ADAMTS13, and TSP-1 proteins were determined in β-thalassemia major patients. A possible association between TSP-1 and vWF and ADAMTS-13 was also evaluated. METHODS: The study was conducted on 80 β-thalassemia major patients and 80 age and sex matched healthy controls. The 80 patients were sub-divided into two groups; splenectomised and non-splenectomised. vWF, ADAMTS13 and TSP-1 plasma level were measured using ELISA technique. RESULTS: There was no significant difference in vWF and TSP-1 levels between patients and controls (p > 0.05). However, ADAMTS13 levels and ADAMTS13 activity/vWF antigen ratio were significantly higher in patients compared to controls (p < 0.05). VWF antigen and TSP-1 level were significantly higher in splenectomised patients (p = 0.025 and p < 0.001, respectively). We also observed a significant decrease in ADAMTS13 activity/vWF antigen ratio among splenectomised compared to non- splenectomised patients (p = 0.019). Correlation analysis showed a significant negative correlation between TSP-1 and vWF Collagen Binding Activity (r = -0.394, p = 0.021) and a positive correlation with ADAMTS13 activity/vWF antigen ratio (r = 0.356, p = 0.039) in splenectomised compared to non- splenectomised patients. CONCLUSION: Our findings highlight the adequacy of patient management protocols for β-TM in Kuwait as patients presented with comparable levels of platelets, vWF and TSP-1 compared to normal controls. The reported increase in ADAMTS13 in patients may be required to maintain normal levels of vWF. Although no active thrombotic episodes were reported at the time of the study, the significant rise in platelets, vWF:Ag and TSP-1 levels in splenectomised patients may indicate a tendency towards hypercoagulability. Monitoring of splenectomised patients is recommended.
简介：在内皮细胞和内皮黏附蛋白（如血管性血友病因子[vWF]）发生改变的情况下，铁幼粒细胞性贫血中的血栓形成倾向起着主要作用。vWF 蛋白的释放导致血管损伤部位的血小板聚集和血栓形成。然后，它被蛋白水解酶 ADAMTS13 降解。血小板反应蛋白-1 是一种多因子糖蛋白，据报道，它与 ADAMTS13 竞争 vWF 蛋白水解的部位。在这项研究中，测定了β-地中海贫血主要患者的 vWF、ADAMTS13 和 TSP-1 蛋白水平。还评估了 TSP-1 与 vWF 和 ADAMTS-13 之间的可能关联。
方法：本研究对 80 名β-地中海贫血主要患者和 80 名年龄和性别匹配的健康对照者进行了研究。80 名患者分为两组；脾切除术和非脾切除术。使用 ELISA 技术测量 vWF、ADAMTS13 和 TSP-1 血浆水平。
结果：患者与对照组之间的 vWF 和 TSP-1 水平无显著差异（p＞0.05）。然而，与对照组相比，ADAMTS13 水平和 ADAMTS13 活性/vWF 抗原比值在患者中显著升高（p＜0.05）。与非脾切除术患者相比，脾切除术患者的 vWF 抗原和 TSP-1 水平显著升高（p=0.025 和 p＜0.001）。我们还观察到，与非脾切除术患者相比，脾切除术患者的 ADAMTS13 活性/vWF 抗原比值显著降低（p=0.019）。相关性分析显示，与非脾切除术患者相比，脾切除术患者的 TSP-1 与 vWF 胶原结合活性呈显著负相关（r=-0.394，p=0.021），与 ADAMTS13 活性/vWF 抗原比值呈显著正相关（r=0.356，p=0.039）。
结论：我们的研究结果强调了科威特β-TM 患者管理方案的充分性，因为与正常对照组相比，患者的血小板、vWF 和 TSP-1 水平相当。患者中报道的 ADAMTS13 增加可能是维持 vWF 正常水平所必需的。尽管在研究时没有报告活跃的血栓形成事件，但脾切除术患者血小板、vWF：Ag 和 TSP-1 水平的显著升高可能表明存在血栓形成倾向。建议监测脾切除术患者。
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