Use and Management of Emicizumab-kxwh (Hemlibra®) for Hemophilia A with and without Inhibitors
Publication Date: April 27, 2022
Last Updated: May 2, 2022
Recommendations
Introduction
- Emicizumab is a recombinant, humanized, bispecific immunoglobulin G4 monoclonal antibody that substitutes for part of the cofactor function of activated factor VIII (FVIIIa) by bridging activated factor IX (FIXa) and factor X (FX). It is indicated for routine prophylaxis to prevent or reduce the frequency of bleeding episodes in adults and children of all ages, newborn and older, with hemophilia A with and without FVIII inhibitors. The medication is given subcutaneously at a loading dose of 3 mg/kg weekly for 4 doses, followed by one of three subsequent maintenance dose regimens: either 1.5 mg/kg weekly, 3 mg/kg every 2 weeks, or 6 mg/kg every 4 weeks. There is significant reduction in annualized bleeding rates at all doses for all age groups, with or without inhibitors. FVIII and emicizumab are fundamentally different proteins and are regulated differently. Some of the differences are show in Table 1.
Title
Use and Management of Emicizumab-kxwh (Hemlibra®) for Hemophilia A with and without Inhibitors
Authoring Organization
National Hemophilia Foundation