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Acquired haemophilia.

C R Hay1

  • 1Manchester Haemophilia Comprehensive Care Centre, University Department of Haematology, Manchester Royal Infirmary, UK.

Bailliere'S Clinical Haematology
|March 31, 1999
PubMed
Summary
This summary is machine-generated.

Acquired haemophilia, a rare bleeding disorder, is treated with immunosuppression to eliminate factor VIII inhibitors and bleeding control. Combination therapy with prednisolone and cyclophosphamide can abolish inhibitors in up to 70% of patients.

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Area of Science:

  • Hematology
  • Immunology
  • Internal Medicine

Background:

  • Acquired haemophilia is a rare, life-threatening bleeding disorder caused by autoimmune depletion of factor VIII.
  • It often affects elderly patients without specific disease associations but can also occur with autoimmune conditions, malignancies, pregnancy, or drug reactions.
  • Equal sex distribution is observed in acquired haemophilia.

Purpose of the Study:

  • To outline the treatment aims for acquired haemophilia, focusing on inhibitor elimination and bleeding management.
  • To review the efficacy of various immunosuppressive and hemostatic agents used in treating acquired haemophilia.

Main Methods:

  • Review of treatment strategies for acquired haemophilia.
  • Analysis of immunosuppressive regimens including prednisolone, cyclophosphamide, azathioprine, vincristine, high-dose immunoglobulin, and cyclosporin A.

Related Experiment Videos

  • Evaluation of hemostatic agents such as porcine factor VIII, recombinant factor VIIa, human factor VIII, and prothrombin complex concentrates.

    • Main Results:

    • Immunosuppression with prednisolone and cyclophosphamide abolishes inhibitors in up to 70% of patients.
    • Other immunosuppressive agents may also be employed.
    • Bleeding can be managed with specific factor concentrates and agents.

    Conclusions:

    • Treatment of acquired haemophilia requires a dual approach: immunosuppression to eradicate the factor VIII inhibitor and hemostatic therapy to control bleeding.
    • Effective management strategies can lead to inhibitor eradication and improved patient outcomes, addressing the primary cause of mortality.