获得性血友病a复发的预后因素来自一项长期观察性研究

IF 3.4 3区医学 Q2 HEMATOLOGY

Research and Practice in Thrombosis and Haemostasis Pub Date : 2025-02-01 DOI:10.1016/j.rpth.2025.102707

Lisa Reich, Florian Gatzke, Steffen Rauchfuss, Stefanie Roth, Wolfgang Miesbach

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引用次数: 0

摘要

获得性血友病A （AHA）是一种罕见的自身免疫性疾病，由抗因子(F)VIII （FVIII）自身抗体引起，可能导致危及生命的出血。虽然已经分析了缓解的预测因素，但缺乏复发的数据。方法研究41例AHA患者的复发预测因素。患者分为两组：复发组（n = 18）和长期稳定缓解组（n = 23），随访至少1年。结果所有复发均发生在首次缓解后1年内。所有纳入患者的中位随访期为3.8年（IQR, 1.8-6.4）。多因素Cox回归分析显示，初始FVIII活性<；1 IU/dL和未能达到初始完全缓解（CR）是复发的重要预测因素。Kaplan-Meier曲线显示，初始FVIII活性为1 IU/dL与≥1 IU/dL的患者的无复发生存率存在显著差异（χ2[1] = 5.950, P = 0.015），初始CR与部分缓解的患者的无复发生存率存在显著差异（χ2[1] = 6.570, P = 0.010）。其他因素，如抑制剂滴度、性别、年龄、世界卫生组织量表、潜在疾病、控制疾病、初始免疫抑制治疗、免疫抑制治疗升级和第21天部分缓解，与复发无显著关系。复发和非复发患者的总生存率无显著差异（χ2[1] = 0.896, P = 0.344）。结论初始FVIII和1iu /dL及未达到初始CR是AHA复发的危险因素。具有这些特征的患者在初次缓解后应密切监测至少1年，因为复发风险增加。

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Prognostic factors for recurrence in acquired hemophilia A-results from a long-term observational study

Objectives

Acquired hemophilia A (AHA) is a rare autoimmune disease caused by autoantibodies against factor (F)VIII (FVIII), potentially leading to life-threatening bleeding. While predictors for remission have been analyzed, data on recurrence is lacking.

Methods

This study investigated predictors of AHA recurrence in 41 patients. Patients were divided into 2 groups: those with recurrence (n = 18) and those in stable long-term remission (n = 23) with at least 1 year of follow-up.

Results

All relapses occurred within 1 year of initial remission. The median follow-up period was 3.8 years (IQR, 1.8-6.4) for all included patients. Multivariate Cox regression analysis revealed that initial FVIII activity <1 IU/dL and failure to achieve initial complete remission (CR) were significant predictors of relapse. Kaplan–Meier curves showed significantly different relapse-free survival rates for patients with initial FVIII activity <1 IU/dL vs ≥1 IU/dL (χ²[1] = 5.950, P = .015), and for those achieving initial CR vs partial remission (χ²[1] = 6.570, P = .010).

Other factors such as inhibitor titer, gender, age, World Health Organization scale, underlying disorder, controlled disorder, initial immunosuppressive therapy, immunosuppressive therapy escalation, and partial remission at day 21 showed no significant relation to recurrences. Overall survival did not differ significantly between relapsing and nonrelapsing patients (χ²[1] = .896, P = .344).

Conclusion

Initial FVIII <1 IU/dL and failure to achieve initial CR are identified as risk factors for recurrence in AHA. Patients with these characteristics should be closely monitored for at least 1 year after initial remission due to increased recurrence risk.

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