Management of factor VIII inhibitors: evolution and current status.
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.
Bloom
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Department of Haematology, University Hospital of Wales, Cardiff, UK.
D000818:Animals; D000933:Antifibrinolytic Agents / Q000627:therapeutic use; D001779:Blood Coagulation Factors / Q000627:therapeutic use; D003131:Combined Modality Therapy; D005164:Factor IX / Q000037:antagonists & inhibitors / Q000276:immunology / Q000627:therapeutic use; D005169:Factor VIII / Q000037:antagonists & inhibitors* / Q000276:immunology / Q000627:therapeutic use; D015942:Factor VIIa / Q000627:therapeutic use; D006467:Hemophilia A / Q000150:complications / Q000276:immunology / Q000628:therapy*; D002836:Hemophilia B / Q000150:complications / Q000276:immunology / Q000628:therapy; D006470:Hemorrhage / Q000209:etiology / Q000628:therapy; D006801:Humans; D007108:Immune Tolerance; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007518:Isoantibodies / Q000276:immunology* / Q000627:therapeutic use; D013552:Swine; D013925:Thromboplastin / Q000627:therapeutic use
D000933:Antifibrinolytic Agents; D001779:Blood Coagulation Factors; D007166:Immunosuppressive Agents; D007518:Isoantibodies; C025667:prothrombin complex concentrates; D005169:Factor VIII; D005164:Factor IX; D013925:Thromboplastin; D015942:Factor VIIa