Supplementary Material for: Plasma Exchange as an Initial Treatment for Severe Bleeding Induced by Acquired Factor V Deficiency: A Case Report and Mini Literature Review

Acquired factor V deficiency (AFVD) is a rare autoimmune bleeding disorder. Unlike acquired hemophilia, bypass therapies with recombinant activated factor VII and activated prothrombin complex concentrates are ineffective for severe bleeding due to AFVD. Although several treatment strategies have been attempted, a standard of care for severe hemorrhage induced by AFVD is lacking. Herein, we report a case of AFVD with severe bleeding that responded to plasma exchange (PE) combined with immunosuppression. We also reviewed previously reported AFVD cases with severe hemorrhage and suggest that PE may be an effective initial treatment for AFVD-induced severe hemorrhage.

获得性凝血因子V缺乏症 (Acquired factor V deficiency, AFVD) 是一种罕见的自身免疫性出血性疾病。与获得性血友病 (Acquired Hemophilia) 不同，针对获得性凝血因子V缺乏症所致的严重出血，采用重组活化凝血因子VII (Recombinant Activated Factor VII) 与活化凝血酶原复合物浓缩物 (Activated Prothrombin Complex Concentrates) 的旁路治疗方案并无疗效。尽管已有多种治疗策略被尝试，但目前仍缺乏针对获得性凝血因子V缺乏症诱发严重出血的标准治疗方案。本文报告1例获得性凝血因子V缺乏症伴严重出血患者，经血浆置换 (Plasma Exchange, PE) 联合免疫抑制治疗后病情得到有效缓解。同时，本文回顾了既往报道的获得性凝血因子V缺乏症严重出血病例，提示血浆置换或可作为获得性凝血因子V缺乏症所致严重出血的有效初始治疗手段。