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Advances in managing rare acquired bleeding disorders.

Massimo Franchini1,2, Giuseppe Marano1, Mario Cruciani1,3

  • 1Italian National Blood Centre, National Institute of Health , Rome, Italy.

Expert Review of Hematology
|April 15, 2020
PubMed
Summary

Rare acquired bleeding disorders, often caused by autoantibodies against clotting factors, present significant bleeding risks. Early diagnosis and specialized care are crucial for effective management and improved patient outcomes.

Keywords:
Acquired hemophilia Aacquired von Willebrand syndromeautoantibodiesbleedingclotting factorstherapy

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Area of Science:

  • Hematology
  • Coagulation Disorders
  • Autoimmune Diseases

Background:

  • Rare acquired bleeding disorders are coagulopathies presenting with spontaneous or trauma-induced hemorrhages.
  • These conditions occur in individuals without a prior personal or family history of bleeding tendencies.

Purpose of the Study:

  • To review rare acquired bleeding disorders caused by autoantibodies against coagulation factors.
  • To discuss the pathogenesis, laboratory findings, clinical features, and management of these conditions.

Main Methods:

  • A Medline/PubMed search was conducted over the past 20 years.
  • The review focuses on autoantibodies against factor VIII (acquired hemophilia A), von Willebrand factor (acquired von Willebrand syndrome), and other factors (V, X, XI, XIII).

Main Results:

  • Autoantibodies against coagulation factors lead to significant bleeding risks.
  • Management is challenging due to severe clinical phenotypes and the need to control bleeding and eliminate autoantibodies.

Conclusions:

  • Early diagnosis of acquired coagulopathies is essential for timely and appropriate treatment.
  • Specialized center support is vital for the correct management of these complex cases to improve patient outcomes.