Earlier the better: convalescent plasma

Abstract

to eradicate inhibitors and how emicizumab may help to reduce the required FVIII dose, which is associated with enormous cost. The observation that FVIII provides additional prohemostatic effects in the presence of emicizumab in the mouse model is therefore noteworthy. 1 Key for any prohemostatic therapy in hemophilia is how the development of hemophilic arthropathy is affected. Early experimental data in monkeys indicate that emicizumab prevents joint bleeds, 8 but clinical trial data indicate that emicizumab does not reduce joint bleeds to 0 in all patients (and neither does FVIII) 2,5-7 ; thus, a better understanding of the effect of emicizumab on the progression and management of hemophilic arthropathy is urgently needed. The emicizumab-adapted hemophilia A mouse model will encompass an important tool to obtain such insights. However, it should be noted that additional modifications are needed, as indicated, before this model is suited for longer-term hemophilic arthropathy studies. 1 Finally, while it is typical to address bleeding in hemophilia from a clottingcentric perspective, it is equally important not to overlook that bleeding, and especially joint bleeding, has its own contributing mechanisms that in addition to coagulation may include endogenous anticoagulant pathways, fibrinolysis, vascular and bone remodeling pathways, and others. Joint bleeding is the cumulative disbalance of these pathways, and the mouse is arguable the best model to test how the contributions of these pathways are affected by emicizumab. The emicizumab-adapted hemophilia A mouse bleeding model developed by Ferri re et al enables such studies and is likely to stimulate new areas of hemophilia A research focused on emicizumab.

Topics & Concepts