Critical Bleeding in Acquired Hemophilia A: Bypassing Agents or Recombinant Porcine Factor VIII?

Tiede, Andreas

doi:10.1055/a-1171-0522

Cited by 9 publications

(5 citation statements)

References 31 publications

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“…Bypassing agents (BPAs), including recombinant factor VIIa (rFVIIa) and activated prothrombin complex concentrate (APCC), as well as recombinant porcine FVIII (rpFVIII, susoctocog alfa) are highly efficacious. 12 However, the risk of bleed recurrence after successful treatment is high, 13 and prophylactic replacement therapy has not been established. Owing to their short half-life, BPAs are not ideal for prophylaxis, although their use has occasionally been reported.…”

Section: A Primer To Ahamentioning

confidence: 99%

Should emicizumab be used in patients with acquired hemophilia A?

Tiede

Kemkes‐Matthes²,

Knöbl

2021

Journal of Thrombosis and Haemostasis

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Emicizumab is currently approved to prevent bleeding in patients with congenital hemophilia A with or without neutralizing antibodies (inhibitors) against factor VIII (FVIII). Here, we present a case‐based discussion of its potential use in acquired hemophilia A (AHA), a severe bleeding disorder caused by autoantibodies against FVIII. State‐of‐the‐art management is based on bypassing agents (recombinant factor VIIa, activated prothrombin complex concentrate) and recombinant porcine FVIII; immunosuppressive therapy (corticosteroids, rituximab, cyclophosphamide) is used to suppress autoantibody formation. Case reports and one series suggest that emicizumab can reduce the risk of bleeding and the requirement for hemostatic therapy until remission of AHA is achieved. Further, it may allow to postpone the start of immunosuppressive therapy or to use less intense regimens. However, the risk‐benefit assessment of emicizumab in AHA is difficult because demographic and clinical characteristics are different compared with congenital hemophilia. Prospective clinical trials are needed before the use of emicizumab can be recommended in AHA.

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Section: A Primer To Ahamentioning

confidence: 99%

Should emicizumab be used in patients with acquired hemophilia A?

Tiede

Kemkes‐Matthes²,

Knöbl

2021

Journal of Thrombosis and Haemostasis

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“…In contrast with congenital hemophilia, because of the high risk of bleeding and the lack of protection from new bleeding until FVIII is over 50%, the hemostatic treatment should be started simultaneously with IST. The choice of the most appropriate hemostatic treatment is usually based on the availability of agents, the anti-porcine titer, costs, monitoring requirements, personal experience and the risk of thrombotic events and safety profile [ 1 , 8 ]. In congenital hemophilia, both plasma-derived and human recombinant FVIII replacement therapy showed efficacy in restoring FVIII activity.…”

Section: Discussion and Literature Reviewmentioning

confidence: 99%

Acquired Hemophilia A Treated with Recombinant Porcine Factor VIII: Case Report and Literature Review on Its Efficacy

et al. 2023

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Acquired hemophilia A (AHA) is a bleeding disorder due to the presence of neutralizing autoantibodies named inhibitors in patients with a previously normal hemostasis. Recent international recommendations suggest the use of bypassing agents or substitutive therapy as the first-line treatment, usually preferring the former. The adequate hemostatic therapy needs an accurate balance between bleeding and thrombotic risks. We report a clinical case of acquired hemophilia A successfully treated with recombinant porcine factor VIII (Susoctocog alfa) as the first-line treatment. Despite the patient having a high-risk thrombotic score and a history of recent myocardial infarction, our experience showed the absence of thrombotic complications related to the use of Susoctocog alfa and a complete restoration of hemostatic parameters. Limited literature is present on the use of recombinant porcine factor VIII as a first-line treatment, and our report supports its use, especially when the thrombotic risk is high.

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“…In contrast with congenital hemophilia, because of the high risk of bleeding and the lack of protection from new bleeding until FVIII is over 50%, the hemostatic treatment should be started simultaneously to IST. The choice of the most appropriate hemostatic treatment is usually based on availability of agents, the anti-porcine titer, costs, monitoring requirements, personal experience, and the risk of thrombotic events and safety profile (1,8). Treatment with both recombinant activated factor VII, activated prothrombin complex concentrate and recombinant porcine factor VIII is associated with a certain risk of arterial and/or venous thrombotic events, especially in elderly patients with risk factors (e.g.…”

Section: Discussionmentioning

confidence: 99%