Succesfull Bypassing Agent Treatment in Intra Cranial Bleeding Hemophilia A Patient with High Titer Inhibitor: a Case Report

Abstract

Intracranial hemorrhage (ICH) is one of the major bleeding  events  causing  mortality  and  long-term  morbidity  in  children with hemophilia, especially those who receive on-demand  therapy. Children with hemophilia have a greater risk of intracranial hemorrhage (ICH) than the general population, with  estimated  prevalences  of  3.5-4%  of  neonates,  and  3-10%  of  children  over  age  1  to  17-year-old  with  hemophilia.The  mortality  rate  of  hemophilia patients with ICH is 20-25%. The development of neutralizing antibodies (inhibitors) against infused factor VIII currently represents the main complication of replacement therapy in patients with severe hemophilia A. Inhibitors, indeed, particularly high-titer inhibitors (>5 BU/mL), greatly complicate the management of bleeding, exposing patients to an increased morbidity and mortality risk, thus representing a significant burden for physicians of Hemophilia Treatment Centers (HTCs). Although bypassing agents (i.e., activated prothrombin complex concentrate [APCC] and recombinant activated factor VII [rFVIIa]) are available for the treatment and prevention of bleeding in inhibitor patients, their efficacy, safety, and cost–benefit outcomes are poorly known in the long term and should be further improved.