Acquired Haemophilia A: An Intriguing Disease

Area of Science:

• Hematology
• Immunology
• Rare Diseases

Background:

• Acquired Haemophilia A (AHA) is a rare autoimmune bleeding disorder.
• It results from neutralizing autoantibodies against Factor VIII (FVIII).
• AHA differs from alloantibodies seen in congenital hemophilia.

Purpose of the Study:

• To summarize the characteristics, diagnosis, and management of Acquired Haemophilia A.
• To highlight the importance of early inhibitor eradication.
• To discuss current therapeutic strategies for bleeding and inhibitor control.

Main Methods:

• Review of clinical presentation and diagnostic criteria for AHA.
• Analysis of incidence, demographics, and etiological factors.
• Overview of treatment modalities including bypassing agents, FVIII concentrates, and immunosuppression.

Main Results:

• AHA affects approximately 1-4 per million people annually, predominantly the elderly.
• Diagnosis relies on prolonged aPTT, normal TT, absence of LA, and inhibitor detection.
• Idiopathic cases represent 44-63%, with others linked to specific factors.

Conclusions:

• Prompt diagnosis and management, including immunosuppressive therapy, are crucial for AHA.
• Effective bleeding control and inhibitor eradication reduce morbidity and mortality.
• Increased awareness, particularly in the elderly, may improve underdiagnosis rates.