虽然众所周知，血友病患者患原发性肝癌（HCC）的年龄比没有此病的患者更低，但关于HCC的临床过程和预后的报道很少。我们的目标是调查HCC和血友病患者的临床过程和预后。我们收纳了在2003年至2021年间最初诊断为HCC的22名血友病患者，并对他们的临床进程和预后进行了回顾性分析。将结果与全国24个HCC病例的随访调查进行比较。所有22名病人均为男性，其中20名病人患有血友病A，2名患者患有血友病B。平均诊断年龄为63岁（范围为45-78岁），低于全国调查报告的平均年龄72岁。最大肿瘤的平均直径为30毫米（范围为11-70毫米），18个肿瘤（82%）在初始诊断时是单发的。所有病例均接受了HCC的标准治疗。进行了61例经动脉化学栓塞治疗、28例射频消融、10例部分切除术和2例放射治疗，并在治疗过程中给了5例病人分子靶向药物治疗。没有与HCC治疗并发症相关的死亡事件。初始治疗后的中位生存时间为6.4年（范围为0.9-18.7年），与全国调查中的中位生存时间5.8年相差不大。HCC的标准治疗能够改善HCC和血友病患者的预后。Copyright © 2023 Wolters Kluwer Health, Inc. 保留所有权利。

Although patients with haemophilia are known to develop hepatocellular carcinoma (HCC) at a lower age than patients without, there are few reports on the clinical course and prognosis of HCC.We aimed to investigate the clinical course and prognosis of patients with HCC and haemophilia.Twenty-two patients with haemophilia, who were initially diagnosed with HCC between 2003 and 2021, were included. Their clinical courses and prognoses were retrospectively analysed. The results were compared with those of the 24th Nationwide Follow-up Survey of Primary Liver Cancer.All 22 patients were male; of these, 20 patients had haemophilia A, and 2 had haemophilia B. The mean age of diagnosis was 63 years (range 45-78 years) which is lower than the mean of 72 years reported in the Nationwide Survey. The mean diameter of the largest tumour was 30 mm (range 11-70 mm), and 18 tumours (82%) were solitary at the initial diagnosis. Standard treatments for HCC were performed in all patients. Sixty-one transarterial chemoembolisation, 28 RFA, 10 hepatectomies, and 2 radiation treatments were performed, and molecular-targeted agents were administered to 5 patients during their clinical courses. No deaths were associated with complications of HCC treatments. The median survival time after initial treatment was 6.4 years (range 0.9-18.7 years) which did not differ much from the median survival time of 5.8 years in the Nationwide Survey.Standard treatment for HCC could improve the prognosis of patients with HCC and haemophilia.Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.