The Japanese Journal of Pediatric Hematology / Oncology
Online ISSN : 2189-5384
Print ISSN : 2187-011X
ISSN-L : 2187-011X
Case Report
Effectiveness of emicizumab in a boy with inhibitor-positive severe hemophilia A
Saori MasudaTakaya HondaMasayoshi YamaokaYoko KatoKagehiro AmanoMasaharu Akiyama
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2020 Volume 57 Issue 5 Pages 399-402

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Abstract

Emicizumab is a bispecific antibody used for hemostasis by binding with activated coagulation factor (F) IX and FX rather than activated FVIII. We report on the case of a 6-year-old boy with inhibitor-positive hemophilia A who has been treated with emicizumab. When he was 9 months old, the patient was found to have severe hemophilia A, for which recombinant (r) FVIII was administered as a replacement therapy. When he was 1 year and 7 months old, he had a severe hematoma due to buttocks bruising, and an FVIII inhibitor was detected. The patient received activated prothrombin complex concentrates as a bypass hemostasis therapy and an rFVIII replacement for immune tolerance induction (ITI). Because the FVIII inhibitor has not disappeared, emicizumab has been administered every two weeks since the patient was 5 years and 4 months old. Emicizumab has had excellent prophylactic effects against bleeding, has reduced injection stress, and has improved the quality of life of patients with ITI failure.

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© 2020 The Japanese Society of Pediatric Hematology / Oncology
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