Prognostic factors for remission of and survival in acquired hemophilia A (AHA): results from the GTH-AH 01/2010 study
Prognostic factors for remission of and survival in acquired hemophilia A (AHA): results from the GTH-AH 01/2010 study
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DOI：
10.1182/blood-2014-07-587089
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发表时间：
2015-02-12
期刊：
影响因子：
20.3
通讯作者：
Knoebl, Paul
中科院分区：
文献类型：
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作者：
Tiede, Andreas;Klamroth, Robert;Knoebl, Paul
获得性血友病A（AHA）是由针对因子VIII（FVIII）的自身抗体引起的。免疫抑制治疗（IST）导致60%至80%的患者在数天至数月内缓解疾病。IST与频繁的不良事件相关，包括感染作为死亡的主要原因。缓解时间的预测因子可以帮助指导IST强度，但尚未建立。我们分析了102例前瞻性入组的采用统一IST方案治疗的患者的预后因素。部分缓解（PR;定义为无活动性出血，FVIII恢复>50 IU/dL，止血治疗停止>24小时），83%的患者在中位31天（范围7-362）后实现。基线FVIII ≥ 1 IU/dL的患者（89%，24天）。校正其他基线特征后，低FVIII仍与较低PR率相关（风险比0.52，95%置信区间0.33-0.81，P
Acquired hemophilia A (AHA) is caused by autoantibodies against factor VIII (FVIII). Immunosuppressive treatment (IST) results in remission of disease in 60% to 80% of patients over a period of days to months. IST is associated with frequent adverse events, including infections as a leading cause of death. Predictors of time to remission could help guide IST intensity but have not been established. We analyzed prognostic factors in 102 prospectively enrolled patients treated with a uniform IST protocol. Partial remission (PR; defined as no active bleeding, FVIII restored>50 IU/dL, hemostatic treatment stopped >24 hours) was achieved by 83% of patients after a median of 31 days (range 7-362). Patients with baseline FVIII = 1 IU/dL (89%, 24 days). After adjustment for other baseline characteristics, low FVIII remained associated with a lower rate of PR (hazard ratio 0.52, 95% confidence interval 0.33-0.81, P