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Published on: June 15, 2020
Venous Malformations and Blood Coagulation in Children
Johanna Aronniemi1,2,3, Satu Långström1,3,4, Katariina A Mattila1,5
1University of Helsinki, Yliopistonkatu 4, 00100 Helsinki, Finland.
Insights
Localized intravascular coagulopathy (LIC) is common in pediatric venous malformations (VMs), with abnormal D-dimer and FXIII levels frequently observed. These findings aid in assessing hemostatic complication risks in children with VMs.
Area of Science:
- Pediatric Hematology
- Vascular Malformations
- Coagulation Disorders
Background:
- Venous malformations (VMs) are congenital vascular anomalies.
- VMs are increasingly linked to coagulation abnormalities, forming localized intravascular coagulopathy (LIC).
- Limited data exists on specific coagulation and fibrinolytic parameters in pediatric VM patients.
Purpose of the Study:
- To systematically analyze coagulation and fibrinolytic parameters in pediatric patients with VMs.
- To correlate laboratory findings with clinical and imaging features of VMs.
- To evaluate the prevalence of LIC in this population.
Main Methods:
- Retrospective analysis of 62 pediatric VM patients.
- Detailed laboratory testing of coagulation and fibrinolytic activity.
- Correlation of laboratory results with clinical and MRI data.
Main Results:
- 39% of patients showed elevated D-dimer and 20% decreased FXIII, indicating LIC.
- LIC markers correlated with larger, deeper, and diffuse VMs.
- Elevated FVIII was associated with smaller, superficial VMs; elevated antithrombin was common but unassociated.
Conclusions:
- Localized intravascular coagulopathy (LIC) is prevalent in pediatric venous malformations.
- Findings provide a basis for risk assessment of hemostatic complications.
- Further research is needed on mechanisms and clinical relations of coagulation disturbances.
Introduction:
Venous malformations (VMs) are congenital low-flow lesions with a wide spectrum of clinical manifestations. An increasing number of studies link VMs to coagulation abnormalities, especially to elevated D-dimer and decreased fibrinogen. This condition, termed localized intravascular coagulopathy (LIC), may pose a risk for hemostatic complications. However, detailed data on the laboratory variables for coagulation and fibrinolytic activity in VM patients are limited. We addressed this question by systematically analyzing the coagulation parameters in pediatric VM patients.
Methods:
We included 62 patients (median age 11.9 years) with detailed laboratory tests for coagulation and fibrinolytic activity at a clinically steady phase. We assessed clinical and imaging features of VMs and their correlations with coagulation and fibrinolysis variables using patient records and MRI.
Results:
D-dimer was elevated in 39% and FXIII decreased in 20% of the patients, as a sign of LIC. Elevated D-dimer and decreased FXIII were associated with large size, deep location, and diffuse and multifocal VMs. FVIII was elevated in 17% of the patients and was associated with small VM size, superficial and confined location, discrete morphology, and less pain. Surprisingly, antithrombin was elevated in 55% of the patients but without associations with clinical or other laboratory variables.
Conclusions:
LIC was common in pediatric patients with VMs. Our results provide a basis for when evaluating the risks of hemostatic complications in children with VMs. Further research is warranted to explore the mechanisms behind coagulation disturbances and their relation to clinical complications.
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