PB1195 - Acquired Hemophilia A: A Report of 5 Cases of Acquired Haemophilia A detected in a tertiary hospital in Northern Malaysia.

Background: Acquired Haemophilia A (AHA) is a rare disease caused by the production of antibodies against coagulation factor VIII. It is associated with high mortality and morbidity, making early diagnosis crucial. Therefore, in our hospital, we implement monitoring the isolated prolonged APTT as the primary screening test to detect cases of Acquired Haemophilia A.

Aims: To highlight the presentation of AHA, management, and outcomes for each patient.

Methods: Retrospective

Results: We reported 5 cases of AHA detected by the lab, each presenting with different clinical settings. Four patients were over the age of 75, while one patient was slightly younger at about 55 years old. All cases presented with a bleeding history, such as prolonged bleeding post procedure, haematuria, extensive bruising, and haemoptysis. The isolated prolonged APTT ranged from around 84-95 seconds. The lab notified the ward of these abnormal APTT results within 24 hours. Mixing tests showed non-correction at 0hr and 2hrs, with inhibitor detected via the Bethesda Assay ranging from 9 to 384BU. The patients were treated with haemostatic agents and immunosuppression. However, only one achieved remission, while the other patients died due to various reasons such as infection, bleeding, and cancer progression.

Conclusion(s): AHA is a potentially life-threatening bleeding disorder, and early diagnosis is crucial for patient outcomes. These cases describe the unusual presentation of patients with AHA and the laboratory's role in the early detection of this condition.