면역관용요법을 시행 중인 A형 혈우병 환자에서 에미시주맙의 예방적 사용요법에 대한 체계적 문헌고찰

Objective: The use of Emicizumab, a novel agent in hemophilia A, in patients with Hemophilia A undergoingimmune tolerance induction (ITI) have been currently evolving in overseas. Here, we aimed to evaluate the real-world useand conceptual study of emicizumab use during ITI in patients with Hemophilia A. Methods: We searched PubMed,Embase, and Cochrane Library databases to perform systematic review according to the Preferred Reporting Items forSystematic reviews and Meta-Analyses (PRISMA). Search terms include ‘hemophilia‘, ‘immune tolerance and‘Emicizumab’. Hemophilia other than hemophilia A, studies with no relevant outcomes, emicizumab use other than duringITI were excluded. Results: Among 419 relevant studies, ten eligible studies were included in this review. Six studiesreported real-world prophylactic use of emicizumab during ITI, and total of nineteen subjects, including overlappingpatients, were evaluated. Other four studies reported conceptual study design of emicizumab in patients with hemophiliaA undergoing ITI. Majority of study set the dose of emicizumab as 1.5mg/kg weekly or 3.0mg/kg bi-weekly after aloading dose of 3.0mg/kg for 4 weeks. In real-world prophylactic use studies, ITI outcome, inhibitor recurrence, antibodytiter, adverse events, and FVIII activity were evaluated as outcomes. Conclusion: This study identified the current statusof emicizumab use and made evidence for the applicability of emicizumab in patients with hemophilia A undergoing ITI. KCI Citation Count: 0