Abstract：
OBJECTIVE: The occurrence of unpredictable pain crises are the principal determinant of the quality of life for patients with venous malformations (VM). A definite coagulation phenomenon, characterized by an increase in D-dimer levels and the presence of phleboliths within the malformation, has been previously reported. By applying Virchow\'s triad and evaluating intralesional samples, our objective is to delineate the coagulation profile and the extent of endothelial dysfunction within the malformation.
METHODS: With the authorization of the Ethics Committee, a research project was undertaken on intralesional and extralesional blood samples from 30 pediatric patients afflicted with spongiform VM. Thromboelastometry analyses were performed using ROTEM Sigma, and the concentration of syndecan-1 was determined by ELISA.
RESULTS: In the ROTEM analyses, the A5, A10, and maximum clot firmness (MCF) values were below the established reference ranges in the intralesional samples in both the EXTEM and INTEM assays, indicating that intralesional clots had significant instability. Furthermore, during the investigation of the delayed fibrinolysis phase using recombinant tissue plasminogen activator (rtPA) in EXTEM analysis, widespread hyperfibrinolysis was observed intralesional. Additionally, analysis of syndecan-1 showed significant differences between extralesional and intralesional levels (p < .026) and controls (p < .03), suggesting differences in the state of endothelium.
CONCLUSIONS: For the first time, we developed a comprehensive understanding of the coagulopathic profile of VM and the role of endothelial dysfunction in its pathogenesis. These findings will enable the implementation of targeted therapies based on the individual coagulation profiles.
METHODS: With the authorization of the Ethics Committee, a research project was undertaken on intralesional and extralesional blood samples from 30 pediatric patients afflicted with spongiform VM. Thromboelastometry analyses were performed using ROTEM Sigma, and the concentration of syndecan-1 was determined by ELISA.
RESULTS: In the ROTEM analyses, the A5, A10, and maximum clot firmness (MCF) values were below the established reference ranges in the intralesional samples in both the EXTEM and INTEM assays, indicating that intralesional clots had significant instability. Furthermore, during the investigation of the delayed fibrinolysis phase using recombinant tissue plasminogen activator (rtPA) in EXTEM analysis, widespread hyperfibrinolysis was observed intralesional. Additionally, analysis of syndecan-1 showed significant differences between extralesional and intralesional levels (p < .026) and controls (p < .03), suggesting differences in the state of endothelium.
CONCLUSIONS: For the first time, we developed a comprehensive understanding of the coagulopathic profile of VM and the role of endothelial dysfunction in its pathogenesis. These findings will enable the implementation of targeted therapies based on the individual coagulation profiles.
摘要：
目的：不可预测的疼痛危机的发生是静脉畸形（VM）患者生活质量的主要决定因素。明确的凝结现象，以D-二聚体水平增加和畸形内存在静脉成分为特征，此前曾有报道。通过应用Virchow的三合会和评估病灶内样本，我们的目标是描述畸形内的凝血特征和内皮功能障碍的程度.
方法：经伦理委员会授权，对30例海绵状VM患儿的病灶内和病灶外血液样本进行了一项研究项目.使用ROTEMSigma进行血栓弹性测量分析，用ELISA法测定syndecan-1的浓度。
结果：在ROTEM分析中，在EXTEM和INTEM测定中，A5、A10和最大凝块硬度(MCF)值均低于病灶内样品的既定参考范围，表明病灶内凝块具有显著的不稳定性。此外，在EXTEM分析中使用重组组织纤溶酶原激活剂（rtPA）研究延迟纤溶阶段，病灶内观察到广泛的高纤维蛋白溶解。此外,syndecan-1的分析显示，病灶外和病灶内水平（p<.026）与对照（p<.03）之间存在显着差异，提示内皮状态的差异。
结论：第一次，我们对VM的凝血病理学特征以及内皮功能障碍在其发病机制中的作用进行了全面的了解.这些发现将使得能够基于个体凝血特征实施靶向治疗。
方法：经伦理委员会授权，对30例海绵状VM患儿的病灶内和病灶外血液样本进行了一项研究项目.使用ROTEMSigma进行血栓弹性测量分析，用ELISA法测定syndecan-1的浓度。
结果：在ROTEM分析中，在EXTEM和INTEM测定中，A5、A10和最大凝块硬度(MCF)值均低于病灶内样品的既定参考范围，表明病灶内凝块具有显著的不稳定性。此外，在EXTEM分析中使用重组组织纤溶酶原激活剂（rtPA）研究延迟纤溶阶段，病灶内观察到广泛的高纤维蛋白溶解。此外,syndecan-1的分析显示，病灶外和病灶内水平（p<.026）与对照（p<.03）之间存在显着差异，提示内皮状态的差异。
结论：第一次，我们对VM的凝血病理学特征以及内皮功能障碍在其发病机制中的作用进行了全面的了解.这些发现将使得能够基于个体凝血特征实施靶向治疗。
