ArticleResearch and practice in thrombosis and haemostasis2021
Emicizumab in tolerized patients with hemophilia A with inhibitors: A single-institution pediatric cohort assessing inhibitor status.
Article in Research and practice in thrombosis and haemostasis, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 9 papers.
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Who cites it
9 citing papers in PubMed, 18 citations in OpenAlex.
- Emicizumab enables rapid tolerance, reducing bleeding during low-dose ITI in pediatric hemophilia A.Blood vessels, thrombosis & hemostasis · 2026Article
- Emicizumab utilization, safety, and outcomes in people with severe hemophilia and no inhibitors: 3-year follow-up. A report from the UK Haemophilia Centre Doctors' Organisation.Research and practice in thrombosis and haemostasis · 2025Article
- Importance of Inhibitor Surveillance During Emicizumab Prophylaxis in Young Children With Hemophilia: An Illustrative Case Series.Case reports in hematology · 2025Article
- Review
- Longitudinal dynamic changes in factor VIII inhibitor titers in patients with hemophilia A and inhibitors receiving emicizumab prophylaxis.International journal of hematology · 2023Article
- Factor VIII inhibitors in hemophilia A treated with emicizumab: longitudinal follow-up of outcomes.Research and practice in thrombosis and haemostasis · 2023Article
- A New Artificial Intelligence Approach Using Extreme Learning Machine as the Potentially Effective Model to Predict and Analyze the Diagnosis of Anemia.Healthcare (Basel, Switzerland) · 2023Article
- Emicizumab state-of-the-art update.Haemophilia : the official journal of the World Federation of Hemophilia · 2022Article
- Research on the Impact of Home Nursing Based on Intelligent Medical Internet of Things on the Quality of Life of Patients with Hemophilia.Computational and mathematical methods in medicine · 2022Article
Corrections and comments
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundThe majority of patients with hemophilia A with inhibitors who undergo immune tolerance induction (ITI) achieve successful tolerance and transition to factor VIII (FVIII) prophylaxis. A portion of these patients have switched to emicizumab for bleeding prevention. However, the risk of inhibitor relapse on emicizumab is unclear.
objectiveTo evaluate the inhibitor status of patients with hemophilia A and inhibitors who achieved successful/partial tolerance after ITI and transitioned from FVIII prophylaxis to emicizumab.
methodsThis is a single-institution, retrospective review of pediatric patients with severe hemophilia A who have completed ITI with FVIII and switched to emicizumab. RESULTS/
conclusionsSeven successfully tolerized and five partially tolerized patients were identified. Three patients continued intermittent FVIII infusions on emicizumab at 50-70 IU/kg twice weekly, once weekly, or every other week due to concerns for inhibitor relapse or loss of recent FVIII tolerance by the treating provider. Eleven of 12 patients (92%) maintained negative inhibitor titers at a mean follow-up of 14.2 ± 6.1 months. One individual had an inhibitor relapse with a peak titer of 2.5 BU/mL. Five of the 11 patients (45%) with negative inhibitor titers had detectable nonneutralizing anti-FVIII IgG4 antibodies, but none of the patients had detectable IgG1 antibodies. There were no inhibitor recurrences in a subset of six patients after FVIII re-exposure for bleeding events or surgery. Given that the presence of an inhibitor significantly impacts factor product choice for bleeding management, ongoing inhibitor monitoring in tolerized patients with hemophilia A who transition to emicizumab is strongly recommended.
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