Literature DB >> 18645138

Association of localized intravascular coagulopathy with venous malformations.

Anne Dompmartin1, Aurélie Acher, Pascal Thibon, Sébastien Tourbach, Cédric Hermans, Véronique Deneys, Ben Pocock, Agnès Lequerrec, Daniel Labbé, Marie-Thérèse Barrellier, Romain Vanwijck, Miikka Vikkula, Laurence M Boon.   

Abstract

OBJECTIVE: To determine which venous malformations (VMs) are at risk for coagulopathy. Venous malformations are slow-flow vascular malformations present at birth, and localized intravascular coagulopathy (LIC) causes pain and thrombosis within a lesion and severe bleeding during surgical procedures.
DESIGN: Prospective convenience sample accrued from 2 multidisciplinary sites in Brussels, Belgium, and Caen, France. PARTICIPANTS: The study population comprised 140 patients with clinical data and coagulation parameters. Magnetic resonance imaging was performed for 110 patients. MAIN OUTCOME MEASURE: Measurement of D-dimer levels.
RESULTS: Of the 140 participants, 59 (42%) showed high D-dimer levels, 36 (61%) of whom had levels higher than 1.0 microg/mL. Six of the participants had low fibrinogen levels. In univariate analysis, large surface, presence of palpable phleboliths, and truncal localization were associated with high D-dimer levels. In the multivariate analysis, only large surface area and presence of phleboliths remained independently associated with high D-dimer levels. Severe LIC, characterized by concomitant low fibrinogen level, was associated with extensive venous malformations of the extremities.
CONCLUSIONS: Localized intravascular coagulopathy is statistically significantly associated with large and/or deep venous malformations that affect any location, which can have a palpable phlebolith. These patients are at risk of local pain due to thrombosis. Lesions with elevated D-dimer levels associated with low fibrinogen levels (severe LIC) commonly affect an extremity and have a high risk of hemorrhage. Low-molecular-weight heparin can be used both to treat the pain caused by LIC and to prevent decompensation of severe LIC to disseminated intravascular coagulopathy.

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Year:  2008        PMID: 18645138      PMCID: PMC5572565          DOI: 10.1001/archderm.144.7.873

Source DB:  PubMed          Journal:  Arch Dermatol        ISSN: 0003-987X


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