Why the study?
Treatment of type 3 von Willebrand disease with VWF and FVIII concentrates may cause anti-VWF antibodies, complicating haemostasis management.
Does emicizumab or other haemostatic strategies improve bleeding control in patients with type 3 von Willebrand disease and anti-VWF inhibitors?
Does emicizumab or other haemostatic strategies improve bleeding control in patients with type 3 von Willebrand disease and anti-VWF inhibitors?
Emicizumab appears to be a viable therapeutic option for managing bleeding in patients with type 3 von Willebrand disease complicated by anti-VWF allo-immunisation.
Answers are grounded in this paper and cite their sources.
Antibody development challenges type 3 VWD haemostasis management; leaves open optimal strategies in this rare complication.
Treatment of type 3 von Willebrand disease by infusion of von Willebrand factor (VWF) and factor VIII (FVIII) concentrates may lead to the development of anti‐VWF antibodies, challenging haemostasis management. The systematic review of the literature presented here retrieved 15 such cases (surgery n = 11, bleeding n = 4). The heterogeneous patient management mostly involved continuous infusion of FVIII, or recombinant FVIIa together with various other strategies. Off‐label infusion of the bispecific monoclonal antibody emicizumab was prescribed in three cases and in a complex local case, ultimately well‐controlled with emicizumab. This illustrates the fact that emicizumab appears as a therapeutic option in this context of allo‐immunisation.
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Briane et al. (2024) studied this question.
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