Hemostasis and Thrombosis Unit, Division of Hematology, Cliniques universitaires Saint-Luc, Université catholique de Louvain, 1200 Brussels, Belgium.
Hemostasis and Thrombosis Unit, Division of Hematology, Cliniques universitaires Saint-Luc, Université catholique de Louvain, 1200 Brussels, Belgium.
Thromb Res. 2018 Aug;168:114-120. doi: 10.1016/j.thromres.2018.06.013. Epub 2018 Jun 18.
Venous malformations (VM) are congenital slow-flow vascular malformations. A common complication is localized intravascular coagulopathy (LIC), which results from chronic, localized, intraluminal activation and consumption of clotting factors and is characterized by elevated D-dimer and, in severe cases, decreased fibrinogen levels. Therapeutic options include anti-coagulation by Low Molecular Weight Heparin (LMWH) which has been reported to improve pain, decrease thrombosis, improve laboratory parameters and reduce hemorrhagic complications. However, the use of subcutaneous injections of LMWH has several limitations and the indications for therapy remain controversial.
We present the first small case series of 3 patients with VM-LIC in whom a long-term treatment by dabigatran etexilate, a direct thrombin inhibitor, was initiated in the setting of ambulatory hemostasis consultations.
Clinically, drug tolerance was excellent in all 3 patients. Moderate hemorrhagic complaints occurred in 2 out of 3 patients but there was no thromboembolic event under dabigatran therapy. Dabigatran provided symptomatic relief on pain, swelling and heaviness feeling. Biologically, dabigatran treatment showed various modifications in D-dimers level but the fibrinogen level and platelet count remained unchanged.
In conclusion, dabigatran etexilate may provide a viable and more convenient alternative to LMWH in the long-term treatment of VM-LIC. The dose has to be tailored in each patient in function of the bleeding tendency and laboratory hemostasis parameters.
静脉畸形(VM)是先天性慢血流血管畸形。常见的并发症是局部血管内凝血紊乱(LIC),这是由于慢性、局部、腔内激活和消耗凝血因子引起的,其特征是 D-二聚体升高,在严重情况下,纤维蛋白原水平降低。治疗选择包括低分子量肝素(LMWH)抗凝,据报道,它可以改善疼痛、减少血栓形成、改善实验室参数和减少出血并发症。然而,皮下注射 LMWH 有几个局限性,治疗指征仍存在争议。
我们介绍了 3 例 VM-LIC 患者的首个小病例系列,这些患者在门诊止血咨询中接受了达比加群酯(一种直接凝血酶抑制剂)的长期治疗。
在所有 3 名患者中,药物耐受性均良好。2 名患者出现中度出血症状,但在达比加群酯治疗期间无血栓栓塞事件。达比加群酯在缓解疼痛、肿胀和沉重感方面有症状缓解作用。在生物学方面,达比加群酯治疗显示 D-二聚体水平的各种变化,但纤维蛋白原水平和血小板计数保持不变。
总之,达比加群酯可能是 VM-LIC 长期治疗中比 LMWH 更可行和更方便的替代药物。剂量必须根据出血倾向和实验室止血参数在每个患者中进行调整。