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Comprehensive Analyses of Coagulation Parameters in Patients with Vascular Anomalies.

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dc.contributor.authorKapp, Friedrich G
dc.contributor.authorSchneider, Cedric
dc.contributor.authorHolm, Annegret
dc.contributor.authorGlonnegger, Hannah
dc.contributor.authorNiemeyer, Charlotte M
dc.contributor.authorRössler, Jochen Karl
dc.contributor.authorZieger, Barbara
dc.date.accessioned2024-10-14T22:53:35Z
dc.date.available2024-10-14T22:53:35Z
dc.date.issued2022-12-08
dc.description.abstractBACKGROUND Vascular anomalies comprise a diverse group of rare diseases with altered blood flow and are often associated with coagulation disorders. The most common example is a localized intravascular coagulopathy in venous malformations leading to elevated D-dimers. In severe cases, this may progress to a disseminated intravascular coagulopathy with subsequent consumption of fibrinogen and thrombocytes predisposing to serious bleeding. A separate coagulopathy is the Kasabach-Merritt phenomenon in kaposiform hemangioendothelioma characterized by platelet trapping leading to thrombocytopenia and eventually consumptive coagulopathy. Our previous work showed impaired von Willebrand factor and platelet aggregometry due to abnormal blood flow, i.e., in ventricular assist devices or extracorporeal membrane oxygenation. With altered blood flow also present in vascular anomalies, we hypothesized that, in particular, the von Willebrand factor parameters and the platelet function may be similarly impacted. METHODS We prospectively recruited 73 patients with different vascular anomaly entities and analyzed their coagulation parameters. RESULTS Acquired von Willebrand syndrome was observed in both of our patients with Kasabach-Merritt phenomenon. In six out of nine patients with complex lymphatic anomalies, both the vWF antigen and activity were upregulated. Platelet aggregometry was impaired in both patients with Kasabach-Merritt phenomenon and in seven out of eight patients with an arteriovenous malformation. CONCLUSIONS The analysis of coagulation parameters in our patients with vascular anomalies advanced our understanding of the underlying pathophysiologies of the observed coagulopathies. This may lead to new treatment options for the, in part, life-threatening bleeding risks in these patients in the future.
dc.description.sponsorshipUniversitätsklinik für Kinderheilkunde
dc.identifier.doi10.48350/176512
dc.identifier.pmid36551267
dc.identifier.publisherDOI10.3390/biom12121840
dc.identifier.urihttps://boris-portal.unibe.ch/handle/20.500.12422/116615
dc.language.isoen
dc.publisherMDPI
dc.relation.ispartofBiomolecules
dc.relation.issn2218-273X
dc.relation.organizationDCD5A442BADAE17DE0405C82790C4DE2
dc.subjectKasabach–Merritt phenomenon coagulopathy localized intravascular coagulopathy platelet function vascular anomalies von Willebrand factor
dc.subject.ddc600 - Technology::610 - Medicine & health
dc.titleComprehensive Analyses of Coagulation Parameters in Patients with Vascular Anomalies.
dc.typearticle
dspace.entity.typePublication
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oaire.citation.issue12
oaire.citation.volume12
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oairecerif.author.affiliationUniversitätsklinik für Kinderheilkunde
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unibe.date.licenseChanged2023-01-10 14:08:21
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unibe.eprints.legacyId176512
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unibe.subtype.articlejournal

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