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Management of rare acquired bleeding disorders
Marzia Menegatti1, Eugenia Biguzzi1, Flora Peyvandi1,2
1Fondazione Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Ca' Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy; and.
Hematology. American Society of Hematology. Education Program
|December 7, 2019
Summary
Autoantibodies targeting clotting factors can cause acquired bleeding disorders, even in healthy individuals. Early diagnosis and specialized treatment are crucial for managing these rare but potentially severe conditions.
Area of Science:
- Hematology
- Immunology
Background:
- Autoantibodies against clotting factors can arise from various conditions, including autoimmune diseases, cancer, drug use, or in seemingly healthy individuals.
- These autoantibodies most frequently target factor VIII (FVIII) or von Willebrand factor, leading to acquired hemophilia A or acquired von Willebrand syndrome.
Observation:
- Rarely, autoantibodies can target other clotting factors like fibrinogen, FII, FV, FVII, FX, FXI, and FXIII.
- Clinical presentations of acquired bleeding disorders vary widely, from mild to life-threatening hemorrhages, often with sudden onset in patients without prior bleeding history.
Findings:
- Delayed diagnosis is possible as some patients lack initial bleeding symptoms.
- Laboratory diagnosis involves screening coagulation tests, factor-level assays, and inhibitor-titrating tests.
Implications:
- Prompt diagnosis of acquired coagulopathies is essential for effective treatment, including managing acute bleeding with bypassing agents and eradicating autoantibodies via immunosuppression.
- Expert intervention at specialized centers is vital for timely recognition and management of these rare bleeding disorders.
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