Bleeding management in type 3 von Willebrand disease with anti-von Willebrand factor inhibitor: A literature review and case report

被引：0

作者：

Briane, Aurelie ^{[1
,2
]}

Horvais, Valerie ^{[3
]}

Sigaud, Marianne ^{[2
,4
]}

Trossaert, Marc ^{[2
,4
]}

Drillaud, Nicolas ^{[2
,4
]}

Ternisien, Catherine ^{[2
,4
]}

Fouassier, Marc ^{[2
,4
]}

Babuty, Antoine ^{[2
,4
]}

机构：

[1] Nantes Univ, CHU Nantes, Serv Med Interne, Nantes, France

[2] Nantes Univ, CHU Nantes, Ctr Ressource & Competence Malad Hemorrag Constitu, Nantes, France

[3] Nantes Univ, CHU Nantes, Unite Invest Clin 17, Nantes, France

[4] Nantes Univ, CHU Nantes, Serv Hematol Biol, Nantes, France

来源：

EJHAEM | 2024年 / 5卷 / 05期

关键词：

allo-antibodies; bleeding disorders; emicizumab; haemostasis; von Willebrand disease; FACTOR-VIII CONCENTRATE; ALLOANTIBODIES;

D O I：

10.1002/jha2.984

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页码：964 / 970

页数：7

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