Acquired hemophilia A: Presenting as a compartment syndrome

Abstract

Acquired hemophilia A (AHA) is a condition caused by autoantibodies (mostly immunoglobulin G) against clotting factor VIII in patients with no prior bleeding disorders. It is usually associated with other autoimmune conditions. Subcutaneous bleed, gastrointestinal bleed, deep muscle bleeds, and mucocutaneous bleedings are some of the common manifestations. Understanding the management principles (control the bleeding, eradication of the inhibitors by the immunosuppression) is prudent as late and inaccurate diagnosis can make it fatal. Here, we present a patient with compartment syndrome due to bleeding into the muscles. Laboratory study was remarkable for prolonged active partial thromboplastin time (aPTT) of 62.9 seconds (normal30-40 seconds), factor VIII (FVIII) activity around 2% (normal is 50%-150%), and high inhibitors levels. Due to concern for AHA, she was treated with steroids and NovoSeven (coagulation factor VIIa, recombinant), leading to stabilization of bleeding. Subsequently, the patient was diagnosed with AHA.