Health‐related Quality of Life in Adults With Haemophilia: A Systematic Review and Meta‐analysis of EQ‐5D and SF‐6D Health‐state Utilities
Wenhui Zhang, Xuewei Li, Xiaolin Ma, Saisai Li, Lingjie Sun, Chunting Zhao, Xue Shi, Wei Wang, Xiaodan LiuAbstract
Introduction
Health‐state utilities (HSUs) summarise health‐related quality of life for economic evaluation. Estimates in haemophilia vary by population, treatment context, instrument, value set, and recruitment setting.
Aim
To synthesise HSU estimates from multi‐attribute utility instruments in adults with haemophilia and evaluate the interpretability of a single pooled value.
Methods
Six databases were searched from inception to 30 June 2026. The primary meta‐analysis included one patient‐reported EQ‐5D or SF‐6D mean, with usable SD, from each independent adult cohort. A REML random‐effects model used Hartung‐Knapp confidence intervals. Prediction intervals, leave‐one‐out analysis, alternative estimators, eligibility and overlap scenarios, and Egger regression assessed robustness.
Results
The review included 35 publications. Sixteen independent adult effects ( n = 3,487) entered the primary analysis. The pooled HSU was 0.675 (95% CI 0.619–0.732). Heterogeneity was substantial (I 2 = 95.4%; τ 2 = 0.0103), with a 95% prediction interval of 0.450–0.900. Leave‐one‐out estimates ranged from 0.668 to 0.690. Egger's intercept was ‐6.24 ( p = 0.003), indicating asymmetry rather than proving publication bias. Data were insufficient for robust subgroup meta‐analysis.
Conclusion
Adults with haemophilia experience a substantial HRQoL burden; however, variability in pooled HSU estimates means that this value should be interpreted as a broad reference point rather than a precise estimate for every setting. Future research would benefit from clearer adult eligibility criteria, more consistent reporting of variance, and broader geographic and population representation, including women and girls with haemophilia and symptomatic carriers.
Cilinical trial Registration information
This systematic review was prospectively registered in PROSPERO (CRD420251234536). Clinical trial registration is not applicable.