Abstract 10162: Acquired Hemophilia A and STEMI: A Management Dilemma

Abstract

Introduction: Thrombotic events are a rare complication of recombinant activated factor VII (rFVIIa) therapy in patients with hemophilia (PWH). We present the case of a patient who developed ST-segment elevation myocardial infarction while receiving rFVIIa replacement therapy for acquired hemophilia A. Case Presentation: A 71-year-old male with non-invasive right ureteral urothelial carcinoma, hypertension, type 2 diabetes mellitus, hyperlipidemia, and non-obstructive coronary artery disease presented for elective robotic-assisted right distal ureterectomy and ureteral reimplantation. He had persistent hematuria after the procedure. Subsequent evaluation for coagulopathy found acquired FVIII deficiency. He received FVIII replacement therapy but failed to respond due to high levels of FVIII inhibitor. His coagulopathy partially improved after treatment with rFVIIa. Unfortunately, the patient developed ST changes on telemetry. 12 lead ECG showed ST elevations in leads II, III, and aVF. Echocardiogram showed hypokinesis of the inferior wall and preserved ejection fraction. A diagnosis of inferior ST segment elevation MI was made; fortunately, the patient was minimally symptomatic. His peak troponin I was 48 ng/mL (normal range < 0.04 ng/mL). After multidisciplinary discussion, rFVIIa was discontinued since it can promote thrombosis. Neither therapeutic anticoagulation nor coronary angiography were pursued due to concerns for bleeding. The patient completed his inferior infarct without complications. His ureteral bleeding also had stopped despite holding rVIIa. Discussion: Acquired hemophilia A (AHA) is a rare entity found in individuals with no history of bleeding disorders in which autoantibodies are directed against clotting factor VIII. rFVIIa is a bypass agent that is approved for acute management of bleeding and perioperative course in AHA. Thrombotic complications are rare, reported in only 0.17% of cases, but its management is challenging. Anticoagulation can be considered depending on bleeding risk. We highlight the importance of an individualized approach with careful risk-benefit assessment and multidisciplinary discussion when managing PWH with acute coronary syndrome.