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

Institutions

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

Controls
|||
ALL TIME
JAN
FEB
MAR
APR
MAY
JUN
JUL
AUG
SEP
OCT
article

Association of Patient-Reported Outcomes with Hemophilia A Inhibitor Status and Treatment Product Type

Megan M. Ullman, Michael B. Nichol, Judith Baker, Nicole Crook et al.
Journal of Clinical Medicine
Hemophilia Treatment and Research
article

Association of Patient-Reported Outcomes with Hemophilia A Inhibitor Status and Treatment Product Type

Megan M. Ullman, Michael B. Nichol, Judith Baker, Nicole Crook, Randall Curtis, Jonathan Roberts, Joanne Wu, Marilyn J. Manco-Johnson
article en

Abstract

<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.

Journal of Clinical MedicineVol. 15(4)
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)
Sanofi, University of Southern California, CSL Behring, University of Texas Health Science Center at Houston, Novo Nordisk, Genentech, University of Colorado Denver
Openalex Percentile: Top 54%
Hemophilia Treatment and Research
AI Navigator

Ask Laika to Summarize, Analyze, and Connect papers live on the map.

Summarize Papers & Methodologies

Extract key findings, datasets, and comparative methods across publications.

Benchmark Rankings & Visual Analytics

Rank top research institutions, authors, funders, topics, and journals by Field-Weighted Citation Impact (FWCI) and paper volume with instant charts.

Connect Distant Disciplines

Bridge topological clusters on the map to find hidden collaborative intersections.