Cerebellar hematoma in severe hemophilia with inhibitor on emicizumab prophylaxis: a case report

Abstract

Background: Emicizumab is a novel prophylactic medication used to treat patients with hemophilia A. It is indicated to minimize the frequency of bleeding episodes and the severity of serious bleeding in patients with hemophilia A utilizing inhibitors. However, some cases of bleeding episodes have been reported, and more data are needed regarding their management and expected outcomes. Case presentation: We report a case of a 4-year-old Saudi Arabian boy with severe hemophilia A who presented with a post-traumatic cerebral hemorrhage. The patient, with high titer inhibitors, was on emicizumab prophylaxis therapy. On hospital admission, he received tranexamic acid (10 mg intravenously, every 6 hours), and recombinant activated factor VII 120 µg/kg every 2 hours for 2 days then every 4 hours for 4 days. On follow-up, the patient showed no signs of neurological deficit. There was no need for emergency neurosurgical intervention since the bleeding had been controlled throughout the first 2 days. There were no recorded thrombotic sequelae or neurological complications, with complete resolution within 10 days. Conclusions: This case implies that low-dose recombinant activated factor VII might be used safely and effectively with patients with hemophilia A on emicizumab prophylaxis, to reduce the risk of cerebral hemorrhage or another episode of serious bleeding along with its long-term complications.