AI Article Synopsis
- - Infusion of von Willebrand factor (VWF) and factor VIII (FVIII) can lead to the development of anti-VWF antibodies, complicating management of bleeding in patients with type 3 von Willebrand disease.
- - A systematic review identified 15 cases where patient management varied, with treatments including continuous FVIII infusion, recombinant FVIIa, and other strategies.
- - Emicizumab, a bispecific monoclonal antibody, was used off-label in three cases and proved effective in managing a complex local case, highlighting its potential as a treatment option for patients with allo-immunisation.
Article Abstract
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 = 11, bleeding = 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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Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11474279 | PMC |
| http://dx.doi.org/10.1002/jha2.984 | DOI Listing |
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