Amano Kagehiro
Department of Laboratory Medicine, Tokyo Medical University.
Department of Molecular Genetics of Coagulation Disorders, Tokyo Medical University.
Rinsho Ketsueki. 2018;59(6):756-763. doi: 10.11406/rinketsu.59.756.
The Japanese Society on Thrombosis and Hemostasis published the guidelines for the treatment of acquired hemophilia A in November 2011 to promote its early diagnosis and appropriate therapy. Successively, the disease was more readily recognized, and a revised version of the guidelines was published in December 2017. Bleeding in patients with acquired hemophilia A is typically more severe than that in patients with congenital hemophilia A. An analysis using the global coagulation assay revealed that the hemostatic function of the plasma of patient with acquired hemophilia A is extremely low, even if factor VIII activities are retained; we present the presumed mechanism for the same. Regarding immunosuppressive therapy to eliminate inhibitors, a large-scale data analysis of patients enrolled in Europe reported that prednisolone (PSL) monotherapy or combination therapy with PSL and cyclophosphamide is the first-line therapy for this disorder, supporting the recommended therapy mentioned in Japan's guidelines. This study aims to highlight the revised guidelines and the latest findings on pathophysiology, diagnosis, and therapy of acquired hemophilia A.
日本血栓与止血学会于2011年11月发布了获得性血友病A的治疗指南，以促进其早期诊断和适当治疗。随后，该病更容易被识别，2017年12月发布了指南的修订版。获得性血友病A患者的出血通常比先天性血友病A患者更严重。一项使用全球凝血试验的分析表明，即使保留了因子VIII活性，获得性血友病A患者血浆的止血功能也极低；我们提出了对此的推测机制。关于消除抑制剂的免疫抑制治疗，对欧洲登记患者的大规模数据分析报告称，泼尼松龙（PSL）单药治疗或PSL与环磷酰胺的联合治疗是该疾病的一线治疗方法，支持日本指南中提到的推荐治疗。本研究旨在强调获得性血友病A的修订指南以及关于其病理生理学、诊断和治疗的最新发现。
