[Hemostatic therapy for hemorrhagic cholecystitis in a patient with hemophilia A with inhibitors].

Abstract

The patient was a 47-year-old man. He was diagnosed with hemophilia A in childhood, and received replacement therapy with FVIII products. However, this became ineffective due to inhibitor production, and he was treated with emicizumab. He developed fever and vomiting, and abdominal CT revealed an enlarged gallbladder and gallstones. Acute cholecystitis was diagnosed, and he was hospitalized and treated conservatively with antibiotics. On the 12th day of illness, the anemia progressed and a high-density area was observed in the gallbladder by CT scan, leading to a diagnosis of hemorrhagic cholecystitis. Since the inhibitor titer was low at 3.6 BU/ml, neutralization therapy with FVIII products was attempted. FVIII activity increased to 155%, and emergency laparoscopic subtotal cholecystectomy was performed successfully. After surgery, FVIII replacement therapy was continued and FVIII activity was maintained, but on the 19th day of illness, it decreased to 16%, and inhibitor levels rose again. Emicizumab is effective in preventing bleeding in hemophilia A with inhibitors, but the risk of bleeding is high if tissue damage leads to severe inflammation or necrosis. Neutralization therapy with FVIII products might be effective in hemorrhagic emergencies and minimally invasive surgery for hemophilia A patients with inhibitors when emicizumab is used to reduce inhibitor levels.