Dompmartin Anne, Acher Aurélie, Thibon Pascal, Tourbach Sébastien, Hermans Cédric, Deneys Véronique, Pocock Ben, Lequerrec Agnès, Labbé Daniel, Barrellier Marie-Thérèse, Vanwijck Romain, Vikkula Miikka, Boon Laurence M
Department of Dermatology, Université de Caen Basse Normandie, Centre Hospitalier Universitaire Caen, Caen, France.
Arch Dermatol. 2008 Jul;144(7):873-7. doi: 10.1001/archderm.144.7.873.
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.
Prospective convenience sample accrued from 2 multidisciplinary sites in Brussels, Belgium, and Caen, France.
The study population comprised 140 patients with clinical data and coagulation parameters. Magnetic resonance imaging was performed for 110 patients.
Measurement of D-dimer levels.
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.
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.
确定哪些静脉畸形（VMs）存在凝血病风险。静脉畸形是出生时就存在的低流量血管畸形，局部血管内凝血病（LIC）会导致病变内疼痛和血栓形成，并在手术过程中引发严重出血。
从比利时布鲁塞尔和法国卡昂的2个多学科地点收集的前瞻性便利样本。
研究人群包括140例具有临床数据和凝血参数的患者。110例患者进行了磁共振成像检查。
D-二聚体水平的测定。
140名参与者中，59名（42%）D-二聚体水平较高，其中36名（61%）高于1.0μg/mL。6名参与者纤维蛋白原水平较低。单因素分析中，大面积、可触及静脉石的存在以及主干定位与高D-二聚体水平相关。多因素分析中，只有大面积和静脉石的存在与高D-二聚体水平独立相关。以纤维蛋白原水平低为特征的严重LIC与四肢广泛的静脉畸形有关。
局部血管内凝血病在统计学上与影响任何部位的大的和/或深部静脉畸形显著相关，这些畸形可能有可触及的静脉石。这些患者有因血栓形成而局部疼痛的风险。D-二聚体水平升高且纤维蛋白原水平低（严重LIC）的病变通常累及一个肢体，并有高出血风险。低分子量肝素可用于治疗LIC引起的疼痛，并预防严重LIC失代偿为弥散性血管内凝血。
