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Acquired haemophilia.

J B Mehta1, S Mehta

  • 1Dr J C Patel Department of Haematology, KEM Hospital, Bombay.

The Journal of the Association of Physicians of India
|December 1, 1990
PubMed
Summary
This summary is machine-generated.

Two cases of acquired hemophilia A, a rare bleeding disorder, were identified. Both patients developed spontaneous bruising due to Factor VIII inhibitors, with limited response to immunosuppressive therapy.

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Area of Science:

  • Hematology
  • Immunology

Background:

  • Acquired hemophilia A (AHA) is a rare autoimmune disorder characterized by the development of inhibitory antibodies against coagulation Factor VIII (F. VIII:C).
  • Unlike inherited hemophilia A, AHA typically affects adults without a prior history of bleeding diathesis.

Purpose of the Study:

  • To describe two cases of acquired hemophilia A presenting with spontaneous bruising.
  • To highlight the challenges in managing AHA, particularly in cases with high antibody titers.

Main Methods:

  • Case report detailing the clinical presentation, diagnostic workup, and treatment of two patients with AHA.
  • Monitoring of Factor VIII inhibitor (anti-F. VIII:C antibody) titers and response to immunosuppressive therapy (prednisolone and cyclophosphamide).

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Main Results:

  • Both patients presented with spontaneous bruising attributed to severe F. VIII:C deficiency caused by autoantibodies.
  • One patient showed no significant reduction in antibody titer after four weeks of combined immunosuppressive therapy.
  • The second patient, with a very high antibody titer, did not receive treatment.

Conclusions:

  • Acquired hemophilia A can manifest as spontaneous bruising in individuals without a prior bleeding history.
  • Treatment of AHA, especially with high antibody titers, may be challenging, with limited response to standard immunosuppressive regimens.