Hayakawa Kana, Tamura Shinobu, Gima Hiroya, Hayakawa Takahiro, Kurihara Toshio, Ooura Maki, Nakano Yoshio, Souri Masayoshi, Ichinose Akitada, Fujimoto Tokuzo
Department of Internal Medicine, Kinan Hospital.
Rinsho Ketsueki. 2014 Nov;55(11):2300-5.
A 62-year-old man had a history of acute aortic dissection (Stanford type A) and had been diagnosed with polycystic kidney disease three years earlier, and then developed end-stage renal failure. He was referred with chief complaints of difficult hemostasis and consecutive hemorrhagic episodes at the puncture site of the shunt soon after dialysis introduction. We suspected chronic disseminated intravascular coagulation (DIC) due to mild thrombocytopenia and a fibrinolytic system abnormality. Plasma factor XIII activity was decreased, but no inhibitor was detected. In addition, contrast-enhanced computed tomography showed exacerbation of a dissecting aortic aneurysm. We finally diagnosed chronic DIC and secondary factor XIII deficiency associated with the aortic aneurysm. We selected treatment involving recombinant human soluble thrombomodulin (rTM) because he was on maintenance dialysis and required long-term follow-up bofore the operation. Hemostatic function improved with regular administration of rTM, and was well-controlled preoperatively.
一名62岁男性有急性主动脉夹层(斯坦福A型)病史,三年前被诊断为多囊肾病,随后发展为终末期肾衰竭。在开始透析后不久,他因分流穿刺部位止血困难和连续出血发作前来就诊。由于轻度血小板减少和纤溶系统异常,我们怀疑是慢性弥散性血管内凝血(DIC)。血浆因子XIII活性降低,但未检测到抑制剂。此外,增强CT显示主动脉夹层动脉瘤加重。我们最终诊断为慢性DIC和与主动脉瘤相关的继发性因子XIII缺乏。由于他正在进行维持性透析且手术前需要长期随访,我们选择了重组人可溶性血栓调节蛋白(rTM)治疗。定期给予rTM后止血功能改善,术前得到良好控制。