Acquired Hemophilia A: A Permanent Challenge for All Physicians

Area of Science:

• Hematology
• Rare Diseases

Background:

• Acquired hemophilia A (AHA) is a rare autoimmune disorder affecting 1.5 per million people in Europe.
• Delayed diagnosis is common, occurring in about one-third of cases, leading to postponed treatment and increased morbidity.
• Spontaneous bleeding affects two-thirds of patients, and AHA carries a 20% mortality rate in symptomatic cases.

Observation:

• AHA diagnosis is typically characterized by a prolonged activated partial thromboplastin time (aPTT) with normal coagulation parameters.
• Confirmation requires detecting antibodies against coagulation Factor VIII (FVIII) and reduced FVIII activity, measured in Bethesda Units.
• This case highlights a patient requiring multiple surgical procedures before AHA diagnosis.

Findings:

• Effective AHA management involves pharmacologic control of bleeding using agents like recombinant activated FVIIa, activated prothrombin complex concentrate, or Emicizumab.
• Simultaneous initiation of immunosuppressive therapy, particularly steroids combined with cyclophosphamide, achieves the highest FVIII antibody eradication rates.
• Potential underlying causes of AHA include neoplasms, autoimmune diseases, and pregnancy.

Implications:

• Early recognition of AHA symptoms and laboratory findings is critical to avoid diagnostic delays.
• Multidisciplinary management strategies are essential for controlling acute bleeding and eradicating FVIII inhibitors.
• Further research into the pathogenesis and optimal treatment of AHA is warranted to improve patient survival and quality of life.