Acquired haemophilia A: Balancing on the edge of haemostasis – Case series
Abstract
Introduction
Emicizumab has recently been investigated for prophylactic use in patients with acquired haemophilia A. While it shows promising results in effectively reducing bleeding episodes, there remains a very delicate question concerning the risk of thromboembolic events. Recently published prospective studies reported thrombotic events in 4% to 8% of cases. However, these studies excluded patients with an increased thrombotic risk or those undergoing treatment for a thromboembolic disease, situations frequently occurring in this elderly patient group. Therefore, we urge clinicians to critically assess which patients would benefit from emicizumab treatment and which would not.
Case series
Here, we present a case series from a Dutch haemophilia treatment centre that highlights the complexity of patients currently managed with acquired haemophilia A. The series discusses individual patient characteristics, underlying comorbidities, and responses to initiated therapy with bypassing agents and immunosuppressive therapy. The pros and cons of emicizumab therapy in these individual patients are discussed.
Discussion
We suggest considering the use of emicizumab in patients with acquired haemophilia A through a personalised approach. This approach involves assessing thrombotic risk on one hand and focusing on controlling bleeding on the other. It should account for the need to rapidly eradicate the inhibitor in cases of acute bleeding necessitating bypassing agents. Alternatively, it should consider the possibility of postponing or reducing immunosuppressive therapy in frail patients without overt bleeding, for whom emicizumab presents an ideal prophylactic option.
© 2025 Marieke J A Verhagen, Sanna R Rijpma, Saskia E M Schols, published by Haemnet Ltd
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