Dompmartin Anne, Ballieux Fanny, Thibon Pascal, Lequerrec Agnès, Hermans Cédric, Clapuyt Philippe, Barrellier Marie-Thérèse, Hammer Franck, Labbé Daniel, Vikkula Miikka, Boon Laurence M
Department of Dermatology, Université de Caen Basse, Normandie, Centre Hospitalier, Universitaire de Caen, Caen, France.
Arch Dermatol. 2009 Nov;145(11):1239-44. doi: 10.1001/archdermatol.2009.296.
To evaluate if elevated D-dimer level is specific for venous malformations (VMs) and thus useful for differential diagnosis, which can be problematic even in specialized interdisciplinary centers. Localized intravascular coagulopathy, characterized by elevated D-dimer levels, has been observed in approximately 40% of patients with VMs.
Prospective convenience sample accrued from 2 interdisciplinary sites.
Two interdisciplinary centers for vascular anomalies in Brussels, Belgium, and Caen, France
The study population comprised 280 patients with clinical data, Doppler ultrasonograms (for 251 patients), and coagulation parameter measurements. Main Outcome Measure Measurement of D-dimer levels.
A VM was diagnosed in 195 of 280 patients (69.6%), and 83 of them had elevated D-dimer levels; the sensitivity of D-dimer dosage was 42.6% (95% confidence interval, 35.6%-49.5%). Among the 85 patients without VM, D-dimer levels were elevated only in 3 patients; the specificity of the dosage was 96.5% (95% confidence interval, 92.5%-100%).
Elevated D-dimer level is highly specific for VMs (pure, combined, or syndromic), and therefore this easy and inexpensive biomarker test should become part of the clinical evaluation of vascular anomalies. It can detect hidden VMs and help differentiate glomuvenous malformation (normal D-dimer levels) from other multifocal venous lesions. Elevated D-dimer level also differentiates a VM from a lymphatic malformation. Moreover, slow-flow Klippel-Trenaunay syndrome (capillaro-lymphatico-venous malformation with limb hypertrophy) can be distinguished from fast-flow Parkes Weber syndrome (capillary malformation with underlying multiple microfistulas and limb hypertrophy). For these reasons, D-dimer level measurement is a useful complementary tool for diagnosing vascular anomalies in everyday practice.
评估D - 二聚体水平升高是否为静脉畸形（VMs）所特有，从而有助于鉴别诊断，即使在专业的多学科中心，鉴别诊断也可能存在问题。在大约40%的VMs患者中观察到以D - 二聚体水平升高为特征的局部血管内凝血病变。
从2个多学科地点收集的前瞻性便利样本。
比利时布鲁塞尔和法国卡昂的两个血管异常多学科中心
研究人群包括280例有临床数据、多普勒超声检查（251例患者）和凝血参数测量值的患者。主要观察指标为D - 二聚体水平的测量。
280例患者中有195例（69.6%）被诊断为VM，其中83例D - 二聚体水平升高；D - 二聚体检测的敏感性为42.6%（95%置信区间，35.6% - 49.5%）。在85例无VM的患者中，仅3例D - 二聚体水平升高；检测的特异性为9...5%（95%置信区间，92.5% - 100%）。
D - 二聚体水平升高对VM（单纯型、复合型或综合征型）具有高度特异性，因此这种简单且廉价的生物标志物检测应成为血管异常临床评估的一部分。它可以检测隐匿性VM，并有助于将球静脉畸形（D - 二聚体水平正常）与其他多灶性静脉病变区分开来。D - 二聚体水平升高还可将VM与淋巴管畸形区分开来。此外，缓慢血流的Klippel - Trenaunay综合征（伴有肢体肥大的毛细血管 - 淋巴管 - 静脉畸形）可与快速血流的Parkes Weber综合征（伴有潜在多发微瘘和肢体肥大的毛细血管畸形）相鉴别。基于这些原因，D - 二聚体水平测量是日常实践中诊断血管异常的有用补充工具。
