Acquired Hemophilia A In Adults: A Multicenter Study from Turkey

Area of Science:

• Hematology
• Autoimmune Diseases
• Rare Diseases

Background:

• Acquired hemophilia A (AHA) is a rare, life-threatening bleeding disorder caused by autoantibodies against factor VIII (FVIII).
• While often idiopathic, AHA can be associated with underlying conditions like pregnancy, malignancy, or infections.
• Effective management requires addressing acute bleeding and implementing immunosuppressive therapy.

Purpose of the Study:

• To investigate the clinical characteristics, management strategies, and survival outcomes of Acquired Hemophilia A patients in Turkey.
• To analyze demographic data, diagnostic parameters, treatment approaches, and complications in a Turkish AHA cohort.
• To provide insights into the long-term prognosis of AHA in a specific national context.

Main Methods:

• A multicenter retrospective study analyzing data from 29 Acquired Hemophilia A patients across eleven Turkish centers.
• Data collected included demographics, FVIII activity, inhibitor titers, bleeding severity, hemostatic and immunosuppressive treatments, and survival status.
• Statistical analysis of aPTT, FVIII, FVIII inhibitor, and hemoglobin levels, mixing tests, and treatment outcomes.

Main Results:

• The median FVIII activity was 0.7% and inhibitor titer was 32.6 BU, with severe bleeding in 44.8% of patients.
• Pregnancy, malignancy, and infections were the most common associated etiologies.
• Most patients (93%) received immunosuppression, with steroid, cyclophosphamide, and rituximab combinations being preferred. Median overall survival was 154.97 months.

Conclusions:

• Acquired Hemophilia A in Turkey presents with varying etiologies and requires prompt hemostatic and immunosuppressive treatment.
• The study highlights the importance of identifying underlying conditions and tailoring treatment for optimal outcomes.
• Turkish AHA patients demonstrate a relatively good long-term survival, underscoring the effectiveness of current management paradigms.