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[Harmless bruises? Coagulopathy despite normal INR].

Ladina Greuter1, Manuela Schöb1, Thomas Lehmann2

  • 1Departement für Innere Medizin, Klinik für Allgemeine Innere Medizin/Hausarztmedizin, Kantonsspital St. Gallen.

Praxis
|July 24, 2014
PubMed
Summary
This summary is machine-generated.

Acquired hemophilia, a rare bleeding disorder, involves antibodies against factor VIII. Treatment focuses on controlling bleeding and suppressing the immune response to the autoantibody.

Keywords:
HemmkörperhämophilieImmunsuppressionacquired haemophiliaactivated prothrombin complexaktivierter Prothrombinkompleximmunosuppressionisolated prolonged aPTTisoliert verlängerte aPTTrecombinant factor VIIarekombinanter Faktor VIIa

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Area of Science:

  • Hematology
  • Immunology
  • Internal Medicine

Background:

  • Acquired hemophilia is a rare autoimmune bleeding disorder.
  • It is characterized by autoantibodies primarily targeting coagulation factor VIII.
  • This condition predominantly manifests as soft tissue and muscular hemorrhages.

Observation:

  • Diagnosis involves identifying a prolonged activated partial thromboplastin time (aPTT).
  • Laboratory findings include the presence of factor VIII antibodies and a reduced factor VIII activity level.
  • Clinical presentation typically involves bleeding into soft tissues and muscles.

Findings:

  • Acute bleeding episodes are managed using bypassing agents like recombinant factor VIIa (NovoSeven®) or activated prothrombin complex (FEIBA®).
  • These agents circumvent the need for factor VIII in the coagulation cascade.
  • Immunosuppressive therapy is crucial for reducing autoantibody levels, commonly involving corticosteroids and cyclophosphamide.

Implications:

  • Treatment strategies aim to both manage active bleeding and eradicate the underlying inhibitor.
  • Rituximab may be added to immunosuppression regimens for refractory cases.
  • Effective management requires a multidisciplinary approach involving hematologists and immunologists.