Hantrakun Nonthakorn, Niprapan Piangrawee, Tuntivate Pakinee, Wongsarikan Nuttanun, Norasetthada Lalita, Tantiworawit Adisak, Rattarittamrong Ekarat, Chai-Adisaksopha Chatree, Rattanathammethee Thanawat, Hantrakool Sasinee, Piriyakhuntorn Pokpong, Punnachet Teerachat
Division of Hematology, Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand.
Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand.
Haemophilia. 2025 May;31(3):412-418. doi: 10.1111/hae.70028. Epub 2025 Mar 18.
Data regarding long-term clinical outcomes in Asian patients with acquired haemophilia A (AHA) was limited.
This study aimed to evaluate the effectiveness of current treatments and their outcomes in a real-world setting among Thai patients with AHA.
This was a retrospective cohort study conducted at a university-based hospital. Patients' characteristics, treatment patterns and disease outcomes were collected. Univariate and multivariate Gray's competing risk analyses were used to examine the factors related to the time to disease response.
From 2009 to 2022, 69 AHA patients with a median age of 68 years (range 36-97) were enrolled. The majority of cases were characterised by the absence of an underlying aetiology (82.6%) and presented as major bleeding (71.0%). As first-line treatment, 79.7% were treated with steroid monotherapy, and 13.0% received a combination of steroid and rituximab. Thirty-one patients (44.9%) received at least one dose of haemostatic agents. After a median time to follow-up of 24.9 months (interquartile range 1.6-78.5), 41 patients (59.4%) attained first disease remission. Factor VIII below 1 IU/dL and the combination of steroid and rituximab were associated with time to disease remission, with subdistribution hazard ratio of 0.3 (95% confidence interval [CI], 0.1-0.7) and 5.2 (95% CI, 2.0-13.4), respectively. The most common complication in this cohort was infection (40.6%).
The combination of steroid and rituximab demonstrated efficacy in the management of AHA. In addition, infectious complications were a significant concern when treating AHA patients.
关于亚洲获得性血友病A（AHA）患者长期临床结局的数据有限。
本研究旨在评估当前治疗方法在泰国AHA患者现实环境中的有效性及其结局。
这是一项在大学附属医院进行的回顾性队列研究。收集了患者的特征、治疗模式和疾病结局。采用单因素和多因素Gray竞争风险分析来检查与疾病缓解时间相关的因素。
2009年至2022年，纳入了69例AHA患者，中位年龄为68岁（范围36 - 97岁）。大多数病例的特征是无潜在病因（82.6%），并表现为严重出血（71.0%）。作为一线治疗，79.7%的患者接受了类固醇单药治疗，13.0%的患者接受了类固醇和利妥昔单抗联合治疗。31例患者（44.9%）接受了至少一剂止血剂。中位随访时间为24.9个月（四分位间距1.6 - 78.5）后，41例患者（59.4%）首次疾病缓解。凝血因子VIII低于1 IU/dL以及类固醇和利妥昔单抗联合治疗与疾病缓解时间相关，亚分布风险比分别为0.3（95%置信区间[CI]，0.1 - 0.7）和5.2（95%CI，2.0 - 13.4）。该队列中最常见的并发症是感染（40.6%）。
类固醇和利妥昔单抗联合治疗在AHA管理中显示出疗效。此外，治疗AHA患者时感染并发症是一个重大问题。
