Immunosuppressive therapy (IST) is administered to patients with acquired hemophilia A (AHA) to eradicate autoantibodies against coagulation factor VIII (FVIII). Data from registries previously demonstrated that IST is often complicated by adverse events, in particular infections. This pilot study was set out to assess the feasibility of reduced-intensity, risk factor–stratified IST. We followed a single-center consecutive cohort of twenty-five patients with AHA receiving IST according to a new institutional treatment standard. Based on results from a previous study, GTH-AH 01/2020, patients were stratified into “poor risk” (FVIII < 1 IU/dl or inhibitor ≥ 20 Bethesda units (BU)/ml) or “good risk” (FVIII ≥ 1 IU/dl and inhibitor < 20 BU/ml). Outcomes were compared between the current cohort and the GTH registry as a historic control (n = 102). Baseline characteristics of the cohort were not different from the historic control. Partial remission, defined as FVIII recovered to > 50 IU/dl, was achieved by 68% of patients after a median time of 112 days, which was lower and significantly later than in the historic control (hazard ratio: 1.8, 95% confidence interval 1.2–2.8). Complete remission, overall survival, and frequency of fatal infections were not different. Grade 3 and 4 infections were more frequent. The impact of risk factors that was observed in the historic cohort was no longer apparent, as partial and complete remission and overall survival were similar in “good risk” and “poor risk” patients. In conclusion, reduced-intensity, risk factor–stratified IST is feasible in AHA but did not decrease the risk of infections and mortality in this cohort.

对患有获得性血友病A（AHA）的患者进行免疫抑制疗法（IST），以根除针对凝血因子VIII（FVIII）的自身抗体。来自注册表的数据以前表明，IST通常因不良事件（特别是感染）而变得复杂。该初步研究旨在评估强度降低，危险因素分层的IST的可行性。根据新的机构治疗标准，我们追踪了一个单中心连续队列的25名接受IST的AHA患者。根据先前研究的结果，GTH-AH 01/2020，将患者分为“风险低”（FVIII <1 IU / dl或抑制剂≥20贝塞斯达单位（BU）/ ml）或“风险高”（FVIII≥ 1 IU / dl和抑制剂<20 BU / ml）。比较了当前队列和GTH注册中心的结果作为历史对照（ñ = 102）。该队列的基线特征与历史对照无差异。68％的患者在中位时间为112天后达到部分缓解（定义为FVIII恢复至> 50 IU / dl），这比历史对照组的患者要低，且显着晚于历史对照组（危险比：1.8，95％置信区间1.2–2.8）。完全缓解，总体生存率和致命感染率没有差异。3级和4级感染更为常见。在历史队列中观察到的危险因素的影响不再明显，因为“好风险”和“低风险”患者的部分和完全缓解以及总体生存率相似。总之，降低强度，危险因素分层的IST在AHA中是可行的，但并未降低该人群的感染风险和死亡率。