Assessing coagulopathy and endothelial dysfunction in paediatric venous
malformation: A thromboelastometry and syndecan-1 study
Abstract
Objective (69) 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 (42) 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 (80) In the ROTEM ®
analyses, the A5, A10, and 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<0.026) and controls (p<0.03), suggesting
differences in the state of endothelium. Conclusions (39) 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.