Atypical acquired hemophilia linked with primary biliary cholangitis: a unique case presentation

Area of Science:

• Hematology
• Immunology
• Gastroenterology

Background:

• Acquired hemophilia A (AHA) is a rare autoimmune disorder characterized by autoantibodies against coagulation Factor VIII.
• It presents with severe bleeding and carries a significant mortality risk.
• While often idiopathic, AHA can be associated with malignancies, pregnancy, drugs, and autoimmune diseases.

Purpose of the Study:

• To report the first documented case of acquired hemophilia A associated with primary biliary cholangitis (PBC).
• To highlight the diagnostic and therapeutic challenges in managing AHA in the context of an autoimmune liver disease.

Main Methods:

• Case presentation of a 48-year-old male with spontaneous oral bleeding post-dental extraction.
• Laboratory investigations including activated partial thromboplastin time (aPTT) and Factor VIII inhibitor assay.
• Etiological workup to identify underlying causes, followed by treatment initiation.

Main Results:

• The patient presented with severe bleeding and a prolonged aPTT, positive for Factor VIII inhibitors.
• Primary biliary cholangitis was identified as the underlying cause.
• Treatment involved a bypassing agent, immunosuppressive therapy, and ursodeoxycholic acid.

Conclusions:

• Acquired hemophilia A can be associated with primary biliary cholangitis, an uncommon but significant comorbidity.
• Early diagnosis and multidisciplinary management are crucial for favorable outcomes in such complex cases.
• This case expands the known associations of AHA and underscores the importance of thorough etiological investigation.