Yasumoto Atsushi, Ishiura Ryohei, Narushima Mitsunaga, Yatomi Yutaka
aDepartment of Clinical Laboratory Medicine, The University of Tokyo HospitalbDepartment of Plastic and Reconstructive Surgery, The University of Tokyo, Tokyo, Japan.
Blood Coagul Fibrinolysis. 2017 Dec;28(8):670-674. doi: 10.1097/MBC.0000000000000666.
: Vascular malformation is occasionally complicated by consumptive coagulopathy, known as localized intravascular coagulopathy (LIC), which is characterized by a reduced fibrinogen level, an elevated D-dimer level and a normal platelet count. We report the case of a 17-year-old Japanese girl who presented with LIC secondary to extensive vascular malformations, whose condition had progressed to disseminated intravascular coagulation (DIC). She suddenly presented with severe anaemia, despite the absence of obvious bleeding, and she began to require regular red blood cell (RBC) transfusions. As she was suffering from paroxysmal atrial fibrillation, we treated her with dabigatran, after obtaining informed consent. Immediately after the administration of dabigatran, the results of clotting tests improved dramatically. Seven months later, she has not required any RBC transfusions, and the dabigatran treatment has been well tolerated. The present case report suggests that dabigatran may be a useful treatment option for patients with DIC associated with vascular malformations.
血管畸形偶尔会并发消耗性凝血病,称为局限性血管内凝血病(LIC),其特征是纤维蛋白原水平降低、D - 二聚体水平升高和血小板计数正常。我们报告一例17岁日本女孩的病例,她因广泛的血管畸形继发LIC,病情已进展为弥散性血管内凝血(DIC)。她突然出现严重贫血,尽管没有明显出血,并且开始需要定期输注红细胞(RBC)。由于她患有阵发性心房颤动,在获得知情同意后,我们用达比加群对她进行治疗。在给予达比加群后,凝血试验结果立即显著改善。七个月后,她不再需要任何RBC输注,并且对达比加群治疗耐受性良好。本病例报告表明,达比加群可能是治疗与血管畸形相关的DIC患者的一种有用治疗选择。