Andreas Tiede1, Paul Giangrande2, Jerome Teitel3, Kagehiro Amano4, Gary Benson5, László Nemes6, Victor Jiménez-Yuste7, Roseline d'Oiron8, Soraya Benchikh El Fegoun9, Craig M Kessler10. 1. Hannover Medical School, Hannover, Germany. 2. University of Oxford, Oxford, UK. 3. St Michael's Hospital, University of Toronto, Toronto, ON, Canada. 4. Tokyo Medical University, Tokyo, Japan. 5. Belfast City Hospital, Belfast, UK. 6. State Health Center, Budapest, Hungary. 7. Hospital Universitario La Paz, Autónoma University, Madrid, Spain. 8. Centre de Référence de l'Hémophilie et des Maladies Hémorragiques Constitutionnelles rares, Hôpitaux Universitaires Paris Sud-APHP-Hôpital Bicêtre, Le Kremlin-Bicêtre, France. 9. Novo Nordisk Health Care AG, Zurich, Switzerland. 10. Georgetown University Medical Center, Washington, DC, USA.
Abstract
BACKGROUND: Acquired haemophilia (AH) is a rare bleeding disorder with significant morbidity and mortality. Most patients initially present to physicians without experience of the disease, delaying diagnosis and potentially worsening outcomes. Existing guidance in AH is limited to clinical opinion of few experts and does not address monitoring bleeds in specific anatomical locations. AIM: Derive consensus from a large sample of experts around the world in monitoring bleeding patients with AH. METHODS: Using the Delphi methodology, a structured survey, designed to derive consensus on how to monitor bleeding patients with AH, was developed by a steering committee for completion by a group of haematologists with an interest in AH. Consensus was defined as ≥75% agreement with a given survey statement. After three rounds of survey refinement, a final list of consensus statements was compiled. RESULTS: Thirty-six global specialists in AH participated. The participants spanned 20 countries and had treated a median of 12.0 (range, 1-50) patients with AH within the preceding 5 years. Consensus was achieved in all items after three survey rounds. In addition to statements on general management of bleeding patients, consensus statements in the following areas were presented: urinary tract, gastrointestinal tract, muscles, skin, joints, nose, pharynx, mouth, intracranial and postpartum. CONCLUSIONS: Here, we present consensus statements derived from a broad sample of global specialists to address monitoring of location-specific bleeds and evaluating efficacy of bleeding treatment in patients with AH. These statements could be applied in practice by treating physicians and validated by individual population surveys.
BACKGROUND:Acquired haemophilia (AH) is a rare bleeding disorder with significant morbidity and mortality. Most patients initially present to physicians without experience of the disease, delaying diagnosis and potentially worsening outcomes. Existing guidance in AH is limited to clinical opinion of few experts and does not address monitoring bleeds in specific anatomical locations. AIM: Derive consensus from a large sample of experts around the world in monitoring bleedingpatients with AH. METHODS: Using the Delphi methodology, a structured survey, designed to derive consensus on how to monitor bleedingpatients with AH, was developed by a steering committee for completion by a group of haematologists with an interest in AH. Consensus was defined as ≥75% agreement with a given survey statement. After three rounds of survey refinement, a final list of consensus statements was compiled. RESULTS: Thirty-six global specialists in AH participated. The participants spanned 20 countries and had treated a median of 12.0 (range, 1-50) patients with AH within the preceding 5 years. Consensus was achieved in all items after three survey rounds. In addition to statements on general management of bleedingpatients, consensus statements in the following areas were presented: urinary tract, gastrointestinal tract, muscles, skin, joints, nose, pharynx, mouth, intracranial and postpartum. CONCLUSIONS: Here, we present consensus statements derived from a broad sample of global specialists to address monitoring of location-specific bleeds and evaluating efficacy of bleeding treatment in patients with AH. These statements could be applied in practice by treating physicians and validated by individual population surveys.
Authors: Katharina Holstein; Xiaofei Liu; Andrea Smith; Paul Knöbl; Robert Klamroth; Ulrich Geisen; Hermann Eichler; Wolfgang Miesbach; Andreas Tiede Journal: Blood Date: 2020-07-16 Impact factor: 22.113
Authors: Andreas Tiede; Peter Collins; Paul Knoebl; Jerome Teitel; Craig Kessler; Midori Shima; Giovanni Di Minno; Roseline d'Oiron; Peter Salaj; Victor Jiménez-Yuste; Angela Huth-Kühne; Paul Giangrande Journal: Haematologica Date: 2020-05-07 Impact factor: 9.941