Hematology Unit, Thrombosis and Hemostasis Reference Regional Center, Università degli studi di Palermo, Palermo, Italy.
Lombardi Comprehensive Cancer Center, Georgetown University Medical Center, Washington, DC, USA.
Haemophilia. 2018 Jan;24(1):43-56. doi: 10.1111/hae.13355. Epub 2017 Sep 27.
There is a paucity of data on the clinical presentation and management of cancer patients with acquired haemophilia (AH), we here report a systematic literature review on acquired haemophilia in the context of cancer.
Treatment outcomes of AH were defined as complete response (CR), partial response (PR) or no response (NR), based on inhibitor eradication, coagulation factor VIII levels and bleeding control. Reported deaths were either related to cancer or bleeding.
Overall, 105 cases were collected and analyzed according to classification of cancer and efficacy of treatments for inhibitor and malignancy. The mean age was 68 years for both males (range 37-86 years) and females (range 43-89 years), 39 patients were female subjects and 66 were males. A solid cancer was diagnosed in 60 subjects, while 45 patients suffered a haematological malignancy. Solid cancers affected mainly males; however, the incidence of solid tumours vs haematological malignancies was not statistically significant (P = .09). Not all patients were treated for their underlying cancer, bleeding and/or inhibitor, in two cases outcome is unavailable. CR was reported in 62.1% (64/103) cases, PR in 9.7% (10/103) cases, NR with or without death was reported in 28.1% (29/103) cases.
CR was best achieved when successful and complete elimination of autoantibodies occurred contemporaneously with the successful treatment of the underlying malignancy. In some cases, recurrent autoantibodies were harbingers of relapsed cancer. Type of cancer, inhibitor titer, treatments administered for bleeding control and inhibitor eradication did not significantly affect clinical outcome of analyzed cases.
获得性血友病（AH）患者的临床表现和治疗数据很少，我们在此报告了癌症相关获得性血友病的系统文献综述。
根据抑制剂消除、凝血因子 VIII 水平和出血控制情况，将 AH 的治疗结果定义为完全缓解（CR）、部分缓解（PR）或无反应（NR）。报告的死亡与癌症或出血有关。
总体而言，根据癌症分类和抑制剂及恶性肿瘤治疗效果，共收集和分析了 105 例病例。男性（年龄范围 37-86 岁）和女性（年龄范围 43-89 岁）的平均年龄均为 68 岁，其中 39 例为女性，66 例为男性。60 例患者诊断为实体瘤，45 例患者患有血液恶性肿瘤。实体瘤主要影响男性；然而，实体瘤与血液恶性肿瘤的发病率无统计学意义（P=0.09）。并非所有患者都接受了潜在癌症、出血和/或抑制剂的治疗，有两例病例的结果无法获得。62.1%（64/103）的病例报告了 CR，9.7%（10/103）的病例报告了 PR，28.1%（29/103）的病例报告了 NR 伴有或不伴有死亡。
当成功消除自身抗体并同时成功治疗潜在恶性肿瘤时，最能达到 CR。在某些情况下，反复出现的自身抗体预示着癌症复发。癌症类型、抑制剂滴度、用于控制出血和消除抑制剂的治疗方法并未显著影响分析病例的临床结果。
