Acquired Hemophilia A: A Review of Recent Data and New Therapeutic Options

Hematology. 2017 Oct;22(9):514-520. doi: 10.1080/10245332.2017.1319115. Epub 2017 Apr 25.

Abstract

Objectives: Acquired hemophilia A (AHA) is a rare, but potentially life-threatening, bleeding disorder caused by an autoantibody against factor VIII that interferes with its coagulant function.

Methods: We performed a narrative review focusing on the diagnostic aspects of AHA and on the current treatment strategies with particular regard to new data and therapeutic developments.

Results: The management of this severe hemorrhagic disorder is based on the control of bleeding with the use of bypassing agents and on the utilization of a variety of immunosuppressant agents with the goal of eliminating the autoantibody permanently.

Conclusion: The optimal management of AHA should be multidisciplinary and requires a close collaboration between physicians from various specialties.

Keywords: Acquired hemophilia A; bleeding; eradication therapy; factor VIII.

Publication types

  • Review

MeSH terms

  • Algorithms
  • Autoantibodies / immunology
  • Blood Coagulation Tests
  • Disease Management
  • Factor VIII / immunology
  • Hemophilia A / diagnosis*
  • Hemophilia A / etiology
  • Hemophilia A / therapy*
  • Hemorrhage / etiology
  • Hemorrhage / therapy
  • Hemostatics / therapeutic use
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Immunosuppressive Agents / therapeutic use

Substances

  • Autoantibodies
  • Hemostatics
  • Immunoglobulins, Intravenous
  • Immunosuppressive Agents
  • Factor VIII

Supplementary concepts

  • Factor 8 deficiency, acquired