Association of Patient-Reported Outcomes with Hemophilia A Inhibitor Status and Treatment Product Type
<b>Objectives:</b> We compared patient-reported outcomes (PROs) in persons with hemophilia A (PwHA) by inhibitor status and prescribed treatment products. <b>Methods:</b> Hematology Utilization Group VIII study enrolled PwHA aged ≥ 2 years to collect PRO data via surveys. A clinical chart review documented the hemophilic severity, inhibitor level and treatment regimen. PROs were compared across inhibitor status and prescribed treatment products. <b>Results:</b> Among 85 enrolled PwHA, 9 (10.6%) had active inhibitors, 22 (25.9%) had tolerized inhibitors, and 54 (63.5%) had no inhibitors. The no-inhibitor group was significantly older (mean: 29.3 ± 13.5 years) than the tolerized (16.3 ± 9.5 years) and active inhibitor (21.9 ± 19.1 years; <i>p</i> = 0.001) groups. A larger proportion of participants with active inhibitors (66.7%) and no inhibitors (53.7%) reported having bleeds in the previous month compared to those with tolerized inhibitors (22.7%, <i>p</i> = 0.02). After covariate adjustment for age and hemophilia severity, the tolerized inhibitor group showed the lowest estimated number of joint bleeds compared to those of the no inhibitor and active inhibitor groups (<i>p</i> = 0.08), and the highest EQ-5D index score (<i>p</i> = 0.09). Emicizumab users reported significantly fewer bleeds in the previous months than those who were prescribed standard or extended half-life factor VIII (33.3% vs. 58.6%, 64.3%, <i>p</i> = 0.04). <b>Conclusions:</b> Participants with active inhibitors experienced joint bleeding rates similar to those of participants without inhibitors, likely attributable to emicizumab use. Tolerized participants reported the fewest joint bleeds and highest quality-of-life scores, potentially reflecting younger age and possible greater prophylaxis adherence. Emicizumab was associated with lower bleed rates compared to standard or extended half-life factor VIII products.
Authors
- Megan M. Ullman (ORCID: https://orcid.org/0000-0002-6621-0137)
- Michael B. Nichol (ORCID: https://orcid.org/0000-0003-4989-9806)
- Judith Baker (ORCID: https://orcid.org/0000-0002-7850-0027)
- Nicole Crook (ORCID: https://orcid.org/0000-0003-1960-7323)
- Randall Curtis (ORCID: https://orcid.org/0000-0002-6859-6432)
- Jonathan Roberts (ORCID: https://orcid.org/0000-0002-9415-9543)
- Joanne Wu
- Marilyn J. Manco-Johnson
Institutions
- University of Southern California (US)
- Illinois College (US)
- Bleeding & Clotting Disorders Institute (US)
- Peoria campus of the University of Illinois System (US)
- Computing Center (RU)
- Center for Inherited Blood Disorders (US)
- University of Colorado Anschutz Medical Campus (US)
- The University of Texas Health Science Center at Houston (US)
Publication Details
- Journal
- Journal of Clinical Medicine
- Published
- 2026-02-14
- DOI
- https://doi.org/10.3390/jcm15041517
- Primary Topic
- Hemophilia Treatment and Research
- Type
- article
- Field-Weighted Citation Impact
- 0.00
Funders
- Sanofi
- University of Southern California
- CSL Behring
- University of Texas Health Science Center at Houston
- Novo Nordisk
- Genentech
- University of Colorado Denver