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Management of Factor VIII Inhibitors: Evolution and Current Status

 

作者: A.L. Bloom,  

 

期刊: Pathophysiology of Haemostasis and Thrombosis  (Karger Available online 1992)
卷期: Volume 22, issue 5  

页码: 268-275

 

ISSN:1424-8832

 

年代: 1992

 

DOI:10.1159/000216334

 

出版商: S. Karger AG

 

关键词: Factor VIII;Factor IV immunoglobulin;Porcine factor VIII;Anti-idiotype;FEIBA;Immunosuppression;Immunological tolerance;Recombinant activated factor VII

 

数据来源: Karger

 

摘要:

Management of patients with factor VIII (and IX) inhibitors includes management of acute bleeds and methods to induce immune suppression and tolerance and to detect patients at risk of developing inhibitors. The methods used over the years to treat acute bleeding have been more or less successful. The best method is to raise factor VIII levels by human or porcine factor VIII concentrate, but this is not usually possible. Prothrombin complex concentrates, activated or non-activated, have enjoyed some success as factor VIII by passing agents, but the development of recombinant activated factor VII represents a new and promising method of inducing haemoslasis at the site of bleeding whilst minimizing the risk of disseminated intravascular coagulation. Alternatively, the use of tissue factor is under consideration to exploit the extrinsic system. Methods to induce immunological tolerance by use of the ‘Bonn’ regime or by the introduction of immunomodulation with the ‘Malmö’ regime of extracorporeal immunodepletion, cyclophosphamide, and intravenous immunoglobulin continue to be attempted with significant but variable success. Gradually the inhibitor problem is being contained, but it is still an important complication of haemophilia therapy.

 

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