Acquired hemophilia A (AHA) is a rare, life-threatening bleeding disorder caused by autoantibodies against coagulation factor VIII (FVIII). Immunosuppressive therapy for AHA aims to arrest bleeding by eliminating FVIII inhibitors. Factor VIII activity overshoot after complete remission (CR) has been reported anecdotally, but details remain unclear. We retrospectively analyzed data from 17 patients with AHA who achieved CR under immunosuppressive therapy between 2009 and 2019 at Gunma University Hospital. FVIII activity overshoot was defined as ≥ 150%. All 17 patients had low FVIII activity (median 2.1%; range < 1.0-8.9%) due to FVIII inhibition (median 14.7 BU/mL; range 2.0-234.0) and all achieved CR within a median of 39 (range 19-173) days. Overshoot occurred in 11 (64.7%) patients and maximal FVIII activity reached > 200% in six of them. The median duration from CR to overshoot was 13 (range 0-154) days. The FVIII overshoot was transient (72.7%) or persistent (27.3%). Venous thromboembolism developed as a complication of overshoot in one patient due to iliac vein compression by a massive hematoma. Overshoot of FVIII activity after CR occurs more frequently than previously expected in patients with AHA.

中文翻译：

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获得性血友病A的患者达到完全缓解的FVIII活性过冲。

获得性血友病A（AHA）是一种罕见的威胁生命的出血性疾病，由针对凝血因子VIII（FVIII）的自身抗体引起。AHA的免疫抑制疗法旨在通过消除FVIII抑制剂来止血。据报道完全缓解（CR）后凝血因子VIII的活性超调，但细节尚不清楚。我们回顾性分析了群马大学医院在2009年至2019年之间通过免疫抑制疗法获得CR的17例AHA患者的数据。FVIII活动超调定义为≥150％。由于FVIII抑制（中位数14.7 BU / mL;范围2.0-234.0），所有17例患者的FVIII活性均较低（中位数为2.1％;范围<1.0-8.9％），并且均在39的中位数内获得了CR（范围19-173）天。11名（64.7％）患者发生过冲，其中六名患者的最大FVIII活性达到> 200％。从CR到超调的平均持续时间为13天（范围为0-154）。FVIII超调是短暂的（72.7％）或持续的（27.3％）。静脉血栓栓塞是一名患者因大血肿oma静脉压迫而引起的过冲并发症。AHA患者发生CR后FVIII活性超调的发生频率比以前预期的高。