Center for Rare Disease and Hemophilia, Taichung Veterans General Hospital, Taichung, Taiwan.
Department of Pediatrics, Taichung Veterans General Hospital, Taichung, Taiwan.
Clin Appl Thromb Hemost. 2019 Jan-Dec;25:1076029619888023. doi: 10.1177/1076029619888023.
There has been an ongoing debate as to whether hemophilia A (HA) is more severe than hemophilia B (HB), and there are studies supporting each side of the argument. The study aimed to investigate whether any differences in major bleeding events exist between patients with severe HA and HB. A nationwide, population-based retrospective cohort study using the National Health Insurance Research Database was conducted. We compared 658 patients with severe HA and 137 patients with severe HB without inhibitors from 1997 to 2013, during the period when adult patients older than 18 years old were treated with the on-demand therapy since birth. There was no significant difference between patients with severe HA and HB in the rate of major bleeding events, with an adjusted relative ratio of 0.79 (95% confidence interval [CI]: 0.36-1.71, = .548). There was also no significant difference in the incidence rate of major bleeding events between adult patients with HA and HB with the on-demand therapy, and an adjusted hazard ratio (HR) of 0.82 (95% CI: 0.65-1.02). However, patients with HA had a lower incidence rate of intracranial hemorrhage, with an adjusted HR of 0.44 (95% CI: 0.25-0.79). In addition, no significant difference in the frequency of major bleeding events requiring hospitalization between patients with HA and HB was found, > .05. In conclusion, the study demonstrated that patients with severe HB encountered a similar rate of major bleeding events to those with severe HA.
有一种持续的争论是关于血友病 A (HA) 是否比血友病 B (HB) 更严重，并且有研究支持这两种观点。本研究旨在调查严重 HA 和 HB 患者之间是否存在主要出血事件的差异。本研究采用基于人群的全国性回顾性队列研究，使用国家健康保险研究数据库。我们比较了 1997 年至 2013 年期间 658 名严重 HA 患者和 137 名无抑制剂的严重 HB 患者，在此期间，18 岁以上的成年患者开始采用按需治疗。严重 HA 和 HB 患者之间主要出血事件的发生率没有显著差异，调整后的相对比值为 0.79（95%置信区间 [CI]：0.36-1.71， =.548）。在按需治疗下，HA 和 HB 的成年患者之间主要出血事件的发生率也没有显著差异，调整后的风险比（HR）为 0.82（95% CI：0.65-1.02）。然而，HA 患者颅内出血的发生率较低，调整后的 HR 为 0.44（95% CI：0.25-0.79）。此外，HA 和 HB 患者因主要出血事件需要住院治疗的频率没有显著差异， >.05。总之，该研究表明，严重 HB 患者发生主要出血事件的频率与严重 HA 患者相似。
