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Tail Vein Transection Bleeding Model in Fully Anesthetized Hemophilia A Mice
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[Acquired hemophilia A].

Kagehiro Amano1,2

  • 1Department of Laboratory Medicine, Tokyo Medical University.

[Rinsho Ketsueki] the Japanese Journal of Clinical Hematology
|July 6, 2018
PubMed
Summary

Acquired hemophilia A patients experience severe bleeding due to low hemostatic function. Revised guidelines and immunosuppressive therapies like prednisolone improve diagnosis and treatment for this rare bleeding disorder.

Keywords:
Acquired hemophilia AGlobal coagulation assayImmunosuppressionInhibitor

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Area of Science:

  • Hematology
  • Internal Medicine

Background:

  • Acquired hemophilia A (AHA) is a rare bleeding disorder characterized by autoantibodies against factor VIII.
  • Bleeding in AHA is often more severe than in congenital hemophilia A.
  • Previous guidelines for AHA treatment were published in 2011, with a revision in 2017.

Purpose of the Study:

  • To highlight the revised 2017 Japanese guidelines for acquired hemophilia A.
  • To present the latest findings on the pathophysiology, diagnosis, and therapy of AHA.
  • To discuss the presumed mechanism of extremely low hemostatic function in AHA patients despite retained factor VIII activity.

Main Methods:

  • Review of revised guidelines published by the Japanese Society on Thrombosis and Hemostasis.
  • Analysis of global coagulation assays to assess hemostatic function in AHA patients.
  • Examination of data on immunosuppressive therapy, including European patient data on prednisolone (PSL) and cyclophosphamide.

Main Results:

  • Global coagulation assay revealed extremely low hemostatic function in AHA plasma, even with retained factor VIII.
  • European data supports first-line immunosuppressive therapy with prednisolone monotherapy or combination therapy with cyclophosphamide.
  • Revised guidelines emphasize early diagnosis and appropriate therapy for acquired hemophilia A.

Conclusions:

  • Acquired hemophilia A requires prompt diagnosis and tailored treatment strategies.
  • Understanding the pathophysiology, including impaired hemostatic function, is crucial for effective management.
  • Current therapeutic approaches, including immunosuppression, align with updated guidelines and international findings.