Disseminated intravascular coagulopathy in a child with extensive venous malformations

Zhuo, Kevin Y; Russell, Susan; Wargon, Orli; Adams, Susan

doi:10.1111/jpc.13460

Cited by 2 publications

(4 citation statements)

References 2 publications

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“…Visceral and joint (intra and/or extra-synovial) VMs present the highest risk of transformation from LIC to DIC 36. Therapeutic interventions such as sclerotherapy and surgery have been documented as triggers for transformation, however there is little quantitative data on the risk of transformation of LIC in VM patients 37–39…”

Section: Resultsmentioning

confidence: 99%

“…36 Therapeutic interventions such as sclerotherapy and surgery have been documented as triggers for transformation, however there is little quantitative data on the risk of transformation of LIC in VM patients. [37][38][39] It is important to distinguish LIC from Kasabach-Merritt syndrome (KMS). This phenomenon is defined as profound thrombocytopenia related to platelet trapping within vascular tumours of infancy, either a kaposiform haemangioendothelioma or a tufted angioma.…”

Section: Hyper-coagulabilitymentioning

confidence: 99%

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Venous malformations: Coagulopathy control and treatment methods

et al. 2020

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Venous malformations (VMs) are ectatic channels which arise as a result of vascular dysmorphogenesis, commonly caused by activating mutations in the endothelial tyrosine kinase receptor (TIE2)/phosphatidylinositol 3-kinase (PI3Kinase) pathway. With a prevalence of 1% in the general population, and a diverse clinical presentation depending on site, size and tissue involvement, their treatment requires a personalised and multidisciplinary approach. Larger lesions are complicated by local intravascular coagulopathy (LIC) causing haemorrhagic and/or thrombotic complications which can progress to disseminated intravascular coagulopathy (DIC). Methods We performed a literature review using a PubMed® search and identified 15 articles to include. References of these texts were examined to further expand the literature review. Principle findings: Several treatment options have been explored, including compression, sclerotherapy, laser therapy, cryoablation and surgery in addition to the management of LIC with low-molecular-weight-heparin (LMWH) and other anticoagulants. Targeted molecular therapies acting on the phosphatidylinositol 3-kinase (PI3Kinase)/Protein Kinase B (AKT)/mammalian target of rapamycin (mTOR) pathway are newly emerging. Conclusion Despite a wealth of literature, larger, multi-centric, randomised and prospective trails are required to offer further clarification on the therapeutic management of coagulopathy control and to provide symptomatic benefit to patients with VMs. There should be efforts to provide long term follow up and to use standardised risk stratification tools and quality of life (QOL) questionnaires to aid comparison of agents and treatment protocols.

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Section: Resultsmentioning

confidence: 99%

Section: Hyper-coagulabilitymentioning

confidence: 99%

Venous malformations: Coagulopathy control and treatment methods

et al. 2020

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“…3,4 In some cases, a decrease in fibrinogen levels may precede the development of disseminated intravascular coagulopathy (DIC), 5,6 although very few cases have been described in the literature. [7][8][9][10] Asymptomatic lesions are common, but they may cause unpredictable pain crises, mostly due to exacerbation of LIC. The emotional distress caused by these episodes can lead to a lower quality of life for patients affected by VM compared with the general population.…”

Section: Introductionmentioning

confidence: 99%

“…This condition can result in elevated D‐dimer levels in extralesional blood, known as localized intralesional coagulopathy (LIC) 3,4 . In some cases, a decrease in fibrinogen levels may precede the development of disseminated intravascular coagulopathy (DIC), 5,6 although very few cases have been described in the literature 7–10 . Asymptomatic lesions are common, but they may cause unpredictable pain crises, mostly due to exacerbation of LIC.…”

Section: Introductionmentioning

confidence: 99%

Assessing coagulopathy and endothelial dysfunction in pediatric venous malformation: A thromboelastometry and syndecan‐1 study

Nava y Hurtado,

Monzon Manzano,

Viana‐Huete

et al. 2024

Pediatric Blood & Cancer

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ObjectiveThe 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.MethodsWith 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.ResultsIn 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.ConclusionsFor 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.

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Disseminated intravascular coagulopathy in a child with extensive venous malformations

Cited by 2 publications

References 2 publications

Venous malformations: Coagulopathy control and treatment methods

Venous malformations: Coagulopathy control and treatment methods

Assessing coagulopathy and endothelial dysfunction in pediatric venous malformation: A thromboelastometry and syndecan‐1 study

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