Immune tolerance induction with mycophenolate-mofetil in two children with haemophilia B and inhibitor.
作者： D. KLARMANN , I. MARTINEZ Saguer , M. B. FUNK , R. KNOEFLER , N. VON HENTIG
DOI: 10.1111/J.1365-2516.2007.01584.X
关键词:
摘要: Summary. Immune tolerance induction (ITI) in haemophilia B patients with inhibitor should be carefully considered because of the relatively poor (25%) overall success rate and high risk complications. ITI combination an immunosuppressive treatment was started two children factor IX (FIX) inhibitor. To avoid anaphylactic reactions boost, FIX replacement therapy stopped received a recombinant activated VII (rFVIIa). After disappearance inhibitor, mycophenolate-mofetil (MMF), dexamethasone (DEXA) intravenous immunoglobulin (IVIG) dose started. could induced patient 2, whereas eradication incomplete 1. Both benefited from immune suppressive tolerated without any allergic Neither development nephrotic syndrome nor severe bleeding episode observed. Strategies to induce inhibitors need explored systematic way. Given low frequency disease even lower incidence inhibitors, prospective randomized studies may not possible. International registry-based retrospective data collection play key role study outcome variables for B.
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