ISSN 0377-9556 (PRINT)
ISSN 2383-9457 (ONLINE)
ISSN 2383-9457 (ONLINE)
Characteristics of the included studies
| Author | Article Type | Title | Protocol | Patient | Observation Period | ITI Dose/Regimen | Emicizumab Dosage | Outcome Mesures |
|---|---|---|---|---|---|---|---|---|
| Batsuli G et al. (2019)8) | Original Article | Immune tolerance induction in paediatric patients with haemophilia A and inhibitors receiving emicizumab prophylaxis | Atlanta protocol | 7 | 4M | 50-100 IU/kg, 3 times per week. starting 4 weeks after initiation of emicizumab. | Loading dose: 3 mg/kg weekly × 4 week, Maintenance dose: 1.5 mg/kg weekly or 3 mg/kg every 2 week | Inhibitor titer |
| Carcao M et al. (2019)9) | Original Article | The changing face of immune tolerance induction in haemophilia A with the advent of emicizumab | FIT protocol | - | - | Start with 50 IU/kg, 3 times per week. If inhibitor titire rises, 200 IU/kg daily. If non-responding after 9 months, product swith and/or start emicizumab. | - | Inhibitor titer |
| Nagao A et al. (2019)10) | Abstract-case study | Immune Tolerance Induction Using Low-dose Fc Fusion Recombinant Factor VIII on Emicizumab in Hemophilia A Patients with Inhibitors, Tokyo Protocol | Tokyo protocol | 3 | - | low- dose (less than 50 IU/kg) extended half-life FVIII 2 times per week. | - | Dose of FVIII and dosing schedule |
| Wieland I et al. (2019)11) | Abstract-case study | Case report: Inhibitor Improvement with Low Dose Immune Tolerance Induction (ITI) with rFVIIIFc and Emicizumab after Failed First Line Therapy with High Dose ITI | Atlanta protocol | 1 | 4Y | 100 IU/kg, 3 times per week. starting 4 weeks after initiation of emicizumab. | Loading dose: 3 mg/kg weekly × 4 week, Maintenance dose: 1.5 mg/kg weekly or 3 mg/kg every 2 week | Inhibitor titer and immunological parameters |
| Batsuli G et al. (2020)12) | Abstract-case study | Updates on the atlanta protocol cohort: Two-year institutional outcomes on immune tolerance induction in children with hemophilia A and inhibitors on emicizumab | Atlanta protocol | 7 | 2Y | 50-100 IU/kg, 3 times per week. starting 4 weeks after initiation of emicizumab. | Loading dose: 3 mg/kg weekly × 4 week, Maintenance dose: 1.5 mg/kg weekly or 3 mg/kg every 2 week | Inhibitor titer |
| Camelo RM et al. (2021)13) | Abstract-case study | Cost-effectiveness analysis of the brazilian immune tolerance induction protocol with recombinant factor VIII and prophylaxis with bypassing agents or emicizumab | BIP protocol | - | - | 50 IU/kg X 3 times per week. upon poor response, increase to 100 IU/kg per day | Loading dose: 3 mg/kg weekly × 4 week, Maintenance dose: 1.5 mg/kg weekly | Ccost-effectiveness analysis |
| Chuansumrit A et al. (2021)14) | Abstract-case study | Effectiveness of monthly low-dose emicizumab prophylaxis without 4-week loading doses among patients with hemophilia a with and without inhibitor: A case series report | - | 1 | 1Y | 100 units/kg of extended half-life factor VIII concentrate 3 times weekly. | whole vial of emicizumab at 30, 75, 105 mg monthly except 3 patients receiving 60 mg monthly which was equal to 1.1 to 1.6 mg/kg of emicizumab | Bleeding rate |
| Garcia J et al. (2021)15) | Brief Report | Real-world case series and summary of current literature of infants and toddlers with severe hemophilia A with inhibitor on prophylaxis with emicizumab | - | 1 | - | 200 units/kg, daily | Loading dose: 3 mg/kg weekly × 4 week, Maintenance dose: 1.5 mg/kg weekly | Bleeding rate |
| Escuriola-Ettingshausen C et al. (2021)16) | Original Article | Design of an international investigatorinitiated study on MOdern Treatment of Inhibitor-positiVe pATiEnts with haemophilia A (MOTIVATE) | MOTIVATE protocol | (120) | (5Y) | Nuwiq® or octanate® or wilate® | - | Inhibitor titer |
| Matsushita T et al. (2022)17) | Original Article | AKATSUKI study: a prospective, multicentre, phase IV study evaluating the safety of emicizumab under and immediately after immune tolerance induction therapy in persons with congenital haemophilia A with factor VIII inhibitors | AKASUSKI protocol | (20) | (193W) | 50 IU/kg, 3 times per week for plasma-derived FVIII. or 2 times per week for recombinant FVIII | Loading dose: 3 mg/kg weekly × 4 week, Maintenance dose: 1.5 mg/kg weekly or 3 mg/kg every 2 week or 6 mg/kg every 4 week | Bleeding rate |
f/u date, follow up date; ITI, immune tolerance induction; M, month; Y, year; FIT, future of immunotolerance treatment; BU, bethesda unit; BIP, brazilian ITI protocol
( ): indicates virtual study design