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Assessing coagulopathy and endothelial dysfunction in pediatric venous malformation: A thromboelastometry and
Francisco Nava Y Hurtado1, Elena Monzon Manzano2,3, Vanesa Viana-Huete4
1Department of Paediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
Pediatric Blood & Cancer
|February 18, 2024
Summary
Venous malformations (VM) cause pain crises due to unstable clots and hyperfibrinolysis. Understanding this coagulopathy and endothelial dysfunction is key to developing targeted therapies for improved patient quality of life.
Area of Science:
- Vascular Biology
- Hematology
- Pediatric Medicine
Background:
- Venous malformations (VM) significantly impair patient quality of life due to unpredictable pain crises.
- A known coagulation abnormality in VM involves elevated D-dimer levels and phleboliths.
- Virchow's triad provides a framework for investigating thrombotic events in VM.
Purpose of the Study:
- To delineate the coagulation profile within VM lesions.
- To assess the extent of endothelial dysfunction in pediatric patients with VM.
- To apply Virchow's triad principles to understand VM pathogenesis.
Main Methods:
- Analysis of intralesional and extralesional blood samples from 30 pediatric VM patients.
- Thromboelastometry (ROTEM Sigma) to evaluate clot kinetics and stability.
- Enzyme-linked immunosorbent assay (ELISA) to measure syndecan-1 levels as a marker of endothelial dysfunction.
Main Results:
- Intralesional samples exhibited significantly unstable clots (low A5, A10, MCF) in both EXTEM and INTEM assays.
- Widespread hyperfibrinolysis was observed in the delayed fibrinolysis phase of intralesional samples.
- Elevated intralesional syndecan-1 levels indicated significant endothelial dysfunction compared to extralesional samples and controls.
Conclusions:
- This study provides the first comprehensive understanding of the coagulopathic profile in VM.
- Endothelial dysfunction plays a crucial role in the pathogenesis of venous malformations.
- Findings pave the way for targeted therapies tailored to individual coagulation profiles in VM patients.
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