Real-World Treatment Patterns and Long-Term Clinical Outcomes in Thai Patients With Acquired Haemophilia A.

INTRODUCTION

Data regarding long-term clinical outcomes in Asian patients with acquired haemophilia A (AHA) was limited.

AIM: This study aimed to evaluate the effectiveness of current treatments and their outcomes in a real-world setting among Thai patients with AHA.

METHODS

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.

RESULTS

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%).

CONCLUSION

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.