Affiliations 

  • 1 Hemostasis and Thrombosis Center, Children's Hospital Los Angeles, University of Southern California Keck School of Medicine, Los Angeles, CA, United States. Electronic address: gyoung@chla.usc.edu
  • 2 Arthur Bloom Haemophilia Centre, Institute of Infection and Immunity, School of Medicine, Cardiff University, Cardiff, United Kingdom
  • 3 Novo Nordisk A/S, Søborg, Denmark
  • 4 Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
  • 5 Department of Hematology, Ogikubo Hospital, Tokyo, Japan
  • 6 Department of Internal Medicine, Carver College of Medicine, University of Iowa, Iowa City, IA, United States
  • 7 Haemophilia Comprehensive Care Centre, Faculty of Health Sciences, NHLS and University of the Witwatersrand, Johannesburg, South Africa
  • 8 Van Creveldkliniek, University Medical Centre, Utrecht, The Netherlands
  • 9 Hôpital Edouard Herriot, University Claude Bernard Lyon 1, Lyon, France
  • 10 Institute of Experimental Haematology and Transfusion Medicine, University Clinic Bonn, Bonn, Germany
  • 11 Erciyes University Medical Faculty, Kayseri, Turkey
  • 12 Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, IRCCS Ca' Granda Foundation, Maggiore Hospital Policlinico, Milan, Italy
  • 13 Haemophilia Center, National Blood Centre, Kuala Lumpur, Malaysia
Thromb Res, 2016 May;141:69-76.
PMID: 26970716 DOI: 10.1016/j.thromres.2016.02.030

Abstract

INTRODUCTION: Paradigm™4 was an international extension trial investigating the safety and efficacy of nonacog beta pegol, a recombinant glycoPEGylated factor IX (FIX) with extended half-life, in haemophilia B patients (FIX activity ≤2%; aged 13-70years) who had previously participated in phase III pivotal (paradigm™2) or surgery (paradigm™3) trials.

METHODS: Patients chose to continue treatment with nonacog beta pegol in either one of two once-weekly prophylaxis arms (10IU/kg or 40IU/kg), or an on-demand arm (40IU/kg for mild/moderate bleeds; 80IU/kg for severe bleeds). The primary objective was to evaluate immunogenicity; key secondary objectives included assessing safety and haemostatic efficacy in the treatment and prevention of bleeds.

RESULTS: Seventy-one patients received prophylaxis or on-demand treatment. No patient developed an inhibitor and no safety concerns were identified. The success rate for the treatment of reported bleeds was 94.6%; most (87.9%) resolved with one injection. The median annualised bleeding rate for patients on prophylaxis was 1.36 (interquartile range [IQR] 0.00-2.23) and 1.00 (IQR 0.00-2.03) for the 10 and 40IU/kg treatment arms, respectively. The mean FIX activity trough achieved for 10 and 40IU once weekly was 9.8% and 21.3%, respectively. Fourteen patients on prophylaxis underwent 23 minor surgical procedures; haemostatic perioperative outcomes for all of those evaluated were 'excellent' or 'good'.

CONCLUSIONS: Nonacog beta pegol showed a favourable tolerability profile (with no safety issues identified) with good prophylactic protection and control of bleeding in previously treated adult and adolescent haemophilia B patients.

* Title and MeSH Headings from MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.

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