PB1197 - Diagnosis and treatment of acquired Hemophilia A at Hematology department in Cho Ray Hospital

Background: Acquired hemophilia A is a rare autoimmune disorder caused by autoantibodies to coagulation factor VIII. AHA patients often bleed spontaneously, severely, difficult to control, and have high treatment costs.

Aims: We present a case series of AHA patients treated at the Hematology department, Cho Ray Hospital from May 2019 to June 2023.

Methods: This is a retrospective case series report of patients who were diagnosed AHA.

Results: 29 patients were included in the study. Mean age was 57.1±16.6 years. Median FVIII activity was 2 U/dL (0–35). Median inhibitor titer was 30.9 BU (1.5–1016.4). Mean Hb was 77.5±19.7 g/L. All patients had at least one bleeding episode before being diagnosed. 21/29 patients had an adverse event. Causes of bleeding: spontaneous (26/29 patients), postoperative (2/29 patients), after injection (1/29 patients). Sites of bleeding: muscular hematomas (20/29 patients), hematuria (7/29 patients), internal bleeding (3/29 patients), cerebral hemorrhage (1 patient). Underlying disorders of AHA: idiopathic (22/29 patients), malignancy (4/29 patients), SLE (3/29 patients). In terms of bleeding treatment, 12 patients received bypass agents including rFVIIa (9 patients) and aPCC (5 patients). The response rate was 91.7%. Thromboembolic events were 0%. All patients received immunosuppressive therapy with corticosteroids alone (19/29 patients), corticosteroids + cyclophosphamide (8/29 patients), corticosteroids + Rituximab (1/29 patients), corticosteroids + MMF (1 patient). Mean hospitalization period: 12.7 days (5-33 days). 11/29 patients developed secondary infections. 3/29 patients perished in the hospital. The mean cost of treatment: 447,130,790, the largest patient cost up to 1,570,965,483.0 VND. Much of the cost is due to the use of bypass agents.

Conclusion(s): From our case series, AHA patients often have severe bleeding, high inhibitor titer, and bypassing agents needed. Although the success rate was high, the cost, hospitalization period, and secondary infection are considered disadvantageous factors. More data is required to find the proper treatment which balances benefits and risks.