Yamada Shinya, Arahata Masahisa, Morishita Eriko, Asakura Hidesaku
Department of Hematology, Kanazawa University Hospital, Kanazawa, Ishikawa, Japan.
Ann Vasc Dis. 2021 Sep 25;14(3):252-255. doi: 10.3400/avd.cr.20-00148.
A 54-year-old Japanese man was diagnosed with blue rubber bleb nevus syndrome (BRBNS) due to venodilation in the lower extremities at birth and gastrointestinal vascular malformations. He also had small bowel bleeding and enhanced-fibrinolytic-type disseminated intravascular coagulation (DIC). Endoscopic sclerotherapy for intestinal hemangioma could not be performed because of bleeding concerns; instead, a combined anticoagulant and antifibrinolytic treatment was performed. Although combination treatment with unfractionated heparin and tranexamic acid proved ineffective for small bowel bleeding, combination treatment with apixaban and tranexamic acid dramatically improved enhanced-fibrinolytic-type DIC. In BRBNS, treatment strategies should be considered after performing detailed coagulation tests.
一名54岁的日本男性因出生时下肢静脉扩张和胃肠道血管畸形被诊断为蓝色橡皮疱痣综合征（BRBNS）。他还出现了小肠出血和纤维蛋白溶解增强型弥散性血管内凝血（DIC）。由于担心出血，无法对肠道血管瘤进行内镜硬化治疗；取而代之的是进行了抗凝和抗纤维蛋白溶解联合治疗。尽管普通肝素和氨甲环酸联合治疗对小肠出血无效，但阿哌沙班和氨甲环酸联合治疗显著改善了纤维蛋白溶解增强型DIC。在BRBNS中，应在进行详细的凝血检查后考虑治疗策略。
