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[Acquired inhibitory body hemophilia]
1Zentrum der Inneren Medizin, Klinikum der Johann-Wolfgang-Goethe-Universität, Frankfurt/M.
Der Anaesthesist
|February 9, 2000
Summary
Acquired haemophilia, caused by autoantibodies against factor VIII or IX, presents a high bleeding risk. Early diagnosis and tailored therapies, including immunosuppression, are crucial for managing this rare bleeding disorder.
Area of Science:
- Hematology
- Immunology
Context:
- Acquired haemophilia is a rare autoimmune disorder characterized by autoantibodies against coagulation factors VIII or IX.
- It affects adults equally across sexes, with increased incidence in older age groups.
Purpose:
- To highlight the diagnostic challenges and therapeutic strategies for acquired haemophilia.
- To differentiate acquired from congenital factor deficiencies and non-specific inhibitors.
Summary:
- Patients present with severe muscle and skin bleeding, unlike the joint bleeds typical of congenital haemophilia.
- Associated conditions trigger autoantibody formation in about half of cases.
- The Bethesda assay quantifies inhibitor titres, guiding treatment selection.
Impact:
- Immediate diagnosis and prompt therapy initiation are essential for improving patient outcomes.
- Available treatments include factor concentrates, recombinant factor VIIa, immunoadsorption, and immunosuppressive therapies.
- Effective management aims to control bleeding and achieve long-term inhibitor reduction or elimination.