Use of thrombin generation assay to personalize treatment of breakthrough bleeds in a patient with hemophilia and inhibitors receiving prophylaxis with emicizumab

Emicizumab prophylaxis among infants and toddlers with severe hemophilia A and inhibitors—a single‐center cohort

“…19 This approach was recently applied to the treatment of breakthrough bleeding in a single patient. 20 Given that emicizumab results in clearly lower aPTT values, a normal or prolonged aPTT result warrants caution. An aPTT assessment may represent a reasonable screening test for clearance or for neutralization of emicizumab by antidrug antibodies.…”

Rescue factor VIII replacement to secure hemostasis in a patient with hemophilia A and inhibitors on emicizumab prophylaxis undergoing hip replacement

“…Its use as prophylactic agent, administered subcutaneously once weekly in patients with hemophilia A and inhibitors aged > 12 years has shown a significant reduction in bleeding frequency compared with previous bypassing therapy. 2,3 Nevertheless breakthrough bleeds may occur during prophylaxis with emicizumab as well as peri-operative bleeding complications, 4,5 and, in those cases, standard bypassing agents (i.e. recombinant activated factor VII, rFVIIa and activated prothrombin complex concentrate (aPCC)) are still needed to control bleeding.…”

Use of global assays to monitor emicizumab prophylactic therapy in patients with haemophilia A with inhibitors

“…5 To our knowledge, there is one published case report and one in vitro study that have proposed using TGA to monitor emicizumab therapy during concomitant FVIII BPA therapy. 8,9 Therefore, to better understand the effects of emicizumab on routine and global coagulation assays, we report herein the results from two patients that received emicizumab prophylaxis. Notable laboratory findings following emicizumab dosing include the spurious "normal" aPTTs in the 20-25 second range, respectively, and the undetermined FVIII levels at both 1 and 2 months following therapy.…”