An Insidious Case of Severe Acquired Factor VIII Inhibitor

Acquired hemophilia occurs when neutralizing antibodies inhibit the activity of coagulation factors, commonly occurring with factor VIII. Most cases are idiopathic; however, autoimmune diseases, certain medications, and malignancies can predispose patients to the development of these inhibitors. Moreover, the initial presentation of the disease is more often catastrophic bleeding, with ecchymosis or mucosal bleeding being less common. This case report outlines an incidental finding of a severe factor VIII inhibitor (with 0% activity) with non-catastrophic bleeding at presentation in the setting of potential lymphoma. Subsequently, the patient was treated with recombinant factor VIIa and immunosuppression with steroids. The case sheds light on the benign presentation of a rapidly fatal disease, thus necessitating urgent and rapid identification. Given the insidious presentation, further research is required on the various factor inhibitors to reduce health costs and improve mortality.