{
  "abstract": "Introduction Despite the known haemostatic action of emicizumab (Hemlibra) in haemophilia A patients, its role in the prevention and control of bleeding in high-demand haemostatic situations, such as major surgery, remains to be determined. Patients receiving regular emicizumab prophylaxis often require concomitant factor VIII (FVIII) therapy during major surgery to prevent uncontrolled bleeding and to promote postoperative healing. However, there are limited prospective surgical data relating to concomitant FVIII and emicizumab use. Simoctocog alfa (Nuwiq) is a B-domain deleted recombinant FVIII produced in a human cell line without chemical modification or protein fusion with proven efficacy as surgical prophylaxis in adult and paediatric patients. The Nuwiq for Perioperative management Of patients With haemophilia A on Emicizumab Regular prophylaxis (NuPOWER) study aims to examine perioperative efficacy and safety of simoctocog alfa in haemophilia A patients on emicizumab prophylaxis undergoing major surgery.Methods and analysis NuPOWER is a prospective, open-label, single-arm, multicentre study that will be conducted at approximately 15 centres worldwide. Up to 28 male patients ≥12 years with severe haemophilia A and no FVIII inhibitors will be recruited. All patients must be receiving regular emicizumab prophylaxis and scheduled to undergo a major surgical procedure during which concomitant simoctocog alfa will be administered. The primary endpoint is the overall haemostatic efficacy of simoctocog alfa, adjudicated by an independent data monitoring committee using a pre-defined algorithm, and will consider intraoperative and postoperative efficacy assessments by the surgeon and investigator, respectively. Secondary endpoints include intraoperative haemostatic efficacy, postoperative haemostatic efficacy, number of allogeneic blood products transfused, perioperative FVIII plasma levels (as measured by FVIII activity) and thrombin generation, and safety parameters. In the era of non-factor therapy, NuPOWER will generate valuable prospective data on concomitant use of simoctocog alfa and emicizumab prophylaxis in patients with severe haemophilia A undergoing major surgery.Ethics and dissemination Ethical approval has been received from institutional review boards/independent ethics committees, and the study will be conducted in compliance with the Declaration of Helsinki. This work will be disseminated by publication of peer-reviewed manuscripts and presentations at scientific meetings.Trial registration number CT EU 2022-502060-21-00; NCT05935358.",
  "authors": [
    {
      "affiliations": [
        "Haematology Research Unit, St. John’s Research Institute, St. John’s National Academy of Health Sciences, Bengaluru, India",
        "Department of Clinical Haematology, St. John’s Medical College Hospital, St. John’s National Academy of Health Sciences, Bengaluru, India"
      ],
      "name": "Alok Srivastava"
    },
    {
      "affiliations": [
        "Haemophilia Centre, St James’s University Hospital, Leeds Teaching Hospitals NHS Trust, Leeds, UK"
      ],
      "name": "Angela Kanny"
    },
    {
      "affiliations": [
        "Haemophilia Centre, University Medical Center Hamburg-Eppendorf, Hamburg, Germany"
      ],
      "name": "Florian Langer"
    },
    {
      "affiliations": [
        "Vascular Medicine and Haemostaseology, Vivantes Klinikum im Friedrichshain, Berlin, Germany"
      ],
      "name": "Cornelia Kubicek-Hofmann"
    },
    {
      "affiliations": [
        "Department of Hematology, Hospital Universitario La Paz, Madrid, Spain"
      ],
      "name": "María Teresa Álvarez Román"
    },
    {
      "affiliations": [
        "Hemophilia Unit, Hospital Universitario Virgen del Rocío, Seville, Andalucía, Spain"
      ],
      "name": "Ramiro Núñez Vázquez"
    },
    {
      "affiliations": [
        "Haemophilia Centre, Department of Haematology, University Hospital Centre Zagreb, Zagreb, Croatia",
        "School of Medicine, University of Zagreb, Zagreb, Croatia"
      ],
      "name": "Ana Boban"
    },
    {
      "affiliations": [
        "Centre for Haemophilia, Institute for Transfusion Medicine, Faculty of Medicine, Ss. Cyril and Methodius University, Skopje, Republic of North Macedonia"
      ],
      "name": "Violeta Dejanova-Ilijevska"
    },
    {
      "affiliations": [
        "Clinic of Haematology, University Clinical Centre of Serbia, Belgrade, Serbia",
        "Faculty of Medicine, University of Belgrade, Belgrade, Serbia"
      ],
      "name": "Predrag Miljić"
    },
    {
      "affiliations": [
        "Department of Pediatrics, Division of Hematology/Oncology, University of Texas Southwestern Medical Center, Dallas, Texas, USA"
      ],
      "name": "Jessica Garcia"
    },
    {
      "affiliations": [
        "Rhine Ruhr Coagulation Centre, Duisburg, Germany"
      ],
      "name": "Susan Halimeh"
    },
    {
      "affiliations": [
        "Haemophilia Treatment Centre, University Hospital of Nantes and French Reference Centre on Haemophilia, Nantes, France"
      ],
      "name": "Nicolas Drillaud"
    },
    {
      "affiliations": [
        "Department of Haemostasis, University Hospital of Tours, Tours, France"
      ],
      "name": "Jean-Baptiste Valentin"
    },
    {
      "affiliations": [
        "IRCCS Humanitas Research Hospital, Center for Thrombosis and Hemorrhagic Diseases, Milan, Italy",
        "Humanitas University, Milan, Italy"
      ],
      "name": "Maria Elisa Mancuso"
    },
    {
      "affiliations": [
        "Center for Bleeding Disorders and Coagulation, Careggi University Hospital, Florence, Italy"
      ],
      "name": "Giancarlo Castaman"
    },
    {
      "affiliations": [
        "Hemostasis and Thrombosis Unit, Azienda Ospedaliero Universitaria Dulbecco, Catanzaro, Italy"
      ],
      "name": "Rita Carlotta Santoro"
    },
    {
      "affiliations": [
        "Coagulation Disorders Unit, Department of Hematology, Comprehensive Cancer Center, Helsinki University Hospital and Helsinki University, Helsinki, Finland"
      ],
      "name": "Anna-Elina Lehtinen"
    },
    {
      "affiliations": [
        "Department of Haematology, Christian Medical College Vellore, Vellore, Tamil Nadu, India"
      ],
      "name": "Aby Abraham"
    },
    {
      "affiliations": [
        "Octapharma USA, Paramus, New Jersey, USA"
      ],
      "name": "Mariko Hashimoto"
    },
    {
      "affiliations": [
        "Octapharma AG, Lachen, Switzerland"
      ],
      "name": "Sigurd Knaub"
    }
  ],
  "title": "NuPOWER (Nuwiq for Perioperative management Of patients With haemophilia A on Emicizumab Regular prophylaxis): protocol for an open-label, single-arm, multicentre study",
  "uid": "233ddf31-e390-5229-b074-15ee690b759b"
}