Diagnosis and Management of Acquired Hemophilia A: A Clinical Analysis from Cho Ray Hospital’s Hematology Department

Background Acquired hemophilia A (AHA) is a rare autoimmune disorder caused by autoantibodies to coagulation factor VIII. Patients with AHA 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 in Cho Ray Hospital from May 2019 to June 2023. Methods : This is a retrospective case series report of patients who were diagnosed with AHA. Demographic information, clinical, laboratory characteristics and treatment were described. This is a retrospective case series report of patients who were diagnosed with AHA. It describes demographic information, clinical and laboratory characteristics, and treatment. Results: 29 patients (males, females) were included in the case series. 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 hemoglobin levels were 77.5 ± 19.7 g/L. All patients had at least one bleeding episode before being diagnosed with which 21/29 patients were an adverse event. The causes of bleeding were spontaneous (26/29 patients), postoperative (2/29 patients) and after injection (1/29 patient). The common site of bleeding were muscular hematomas (20/29 patients), hematuria (7/29 patients), internal bleeding (3/29 patients) and cerebral hemorrhage (1 patient). The underlying disorders of AHA were idiopathic (22/29 patients), malignancy (4/29 patients) and SLE (3/29 patients). In terms of bleeding treatment, 12 patients received bypass agent 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 combined with cyclophosphamide (8/29 patients), corticosteroids combined with Rituximab (1/29 patients), and corticosteroids combined with MMF (1 patient). Mean hospitalization period was 12.7 days (5-33 days). 11/29 patients developed secondary infections. 3/29 patients perished in the hospital. Conclusion : 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.