Association of localized intravascular coagulopathy with venous malformations.
Association of localized intravascular coagulopathy with venous malformations.
复制标题
局部血管内凝血病与静脉畸形的关联。
DOI：
10.1001/archderm.144.7.873
复制
发表时间：
2008-07
影响因子：
--
通讯作者：
Boon LM
中科院分区：
文献类型：
--
作者：
Dompmartin A;Acher A;Thibon P;Tourbach S;Hermans C;Deneys V;Pocock B;Lequerrec A;Labbé D;Barrellier MT;Vanwijck R;Vikkula M;Boon LM
静脉畸形（VM）是出生时存在的血流缓慢的血管畸形。局部血管内凝血病（LIC）会导致疼痛和病变内血栓形成以及外科手术期间的严重出血。本研究旨在确定哪些静脉畸形有凝血功能障碍的风险。前瞻性便利样本来自比利时布鲁塞尔和法国卡昂的2个多学科研究中心。140例患者的临床数据和凝血参数。对110例患者进行了磁共振成像。42%的患者显示出高D-二聚体，其中61%的D-二聚体超过1000 ng/ml。其中6例纤维蛋白原水平较低。在单变量分析中，大表面，存在可触及的静脉结石，和躯干定位与高D-二聚体水平。在多变量分析中，只有大表面积和静脉石的存在与高D-二聚体水平独立相关。严重的LIC，其特征是伴随低纤维蛋白原，与广泛的四肢VM相关。局部血管内凝血病与影响任何部位的大静脉和/或深静脉畸形（可触及静脉结石）有统计学相关性。这些患者有因血栓形成引起局部疼痛的风险。与低纤维蛋白原水平相关的D-二聚体水平升高的病变（严重LIC）通常影响肢体，并且具有高出血风险。低分子量肝素既可用于治疗LIC引起的疼痛，也可用于预防严重LIC失代偿至DIC。
Venous malformations (VM) are slow-flow vascular malformations present at birth. Localized intravascular coagulopathy (LIC) causes pain and thrombosis within a lesion and severe bleeding during surgical procedures. This study aimed to determine which venous malformations are at risk for coagulopathy. Prospective convenience sample accrued from 2 multidisciplinary sites in Brussels, Belgium and Caen, France. 140 patients with clinical data and coagulation parameters. Magnetic resonance imaging was performed for 110 patients. Forty two per cent of patients showed high D-dimers, 61% of which had more than 1000ng/ml. Six of them 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, was associated with extensive VM of extremities. Localized intravascular coagulopathy is statistically 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 DIC.
登录
查看更多内容
DOI：
10.1097/01.moo.0000145946.67222.01
发表时间：
2004-12-01
影响因子：
1.6
作者：
Buckmiller, Lisa M
通讯作者：
Buckmiller, Lisa M
DOI：
10.1016/j.anplas.2006.07.018
发表时间：
2006-08-01
期刊：
Annales de chirurgie plastique et esthetique
影响因子：
--
作者：
Casanova, D;Boon, L-M;Vikkula, M
通讯作者：
Vikkula, M
影响因子：
3.6
作者：
Sarkar, M;Mulliken, JB;Burrows, PE
通讯作者：
Burrows, PE
影响因子：
--
作者：
Boon, LM;Mulliken, JB;Vikkula, M
通讯作者：
Vikkula, M
影响因子：
158.5
作者：
EZEKOWITZ, RAB;MULLIKEN, JB;FOLKMAN, J
通讯作者：
FOLKMAN, J