Identifying Nonresponsive Bleeding Episodes in Patients With Haemophilia and Inhibitors: A Consensus Definition

• Published in: Haemophilia : the official journal of the World Federation of Hemophilia Vol. 17; no. 1
• Main Authors: Berntorp, Erik, Collins, Peter, d'Oiron, Roseline, Ewing, Nadia, Gringeri, Alessandro, Negrier, Claude
• Format: Journal Article
• Language: English
• Published: Wiley 02.09.2010
• Subjects: Medicine
• ISSN: 1351-8216; 1365-2516
• DOI: 10.1111/j.1365-2516.2010.02377.x

Introduction: Assessing response to treatment with bypassing agents presents a substantial challenge in the treatment of patients with haemophilia and inhibitors. Rapid and accurate identification of bleeding episodes that are nonresponsive to bypassing therapy with either Factor Eight Inhibitor Bypassing Activity (FEIBA; Baxter AG, Vienna, Austria) or recombinant activated factor VII (rFVIIa; NovoSeven®, Novo Nordisk A/S, Bagsvaerd, Denmark) is essential to guide treatment decisions and optimise patient outcomes through early intervention. Although both bypassing agents are effective, differential responses to therapy necessitate multiple therapeutic options. Aim: This paper provides a consensus definition for non-life-threatening joint and muscle bleeds that are nonresponsive to bypassing agents. Methods: An international panel of 7 physicians met in December 2008 to develop the consensus definition using a modified National Institutes of Health Consensus Development Conference method. Results: The consequent definition of non-life-threatening bleeding episodes that are nonresponsive to bypassing treatment provides a global picture of the condition of the patient during such an event. Identification of nonresponsiveness is based on various criteria: pain, swelling/tension, mobility, patient perception, and laboratory parameters. Criteria can be assessed subjectively by the patient/parent, and/or objectively by the clinician. Although the precise timing of each determination should be at the discretion of the physician, bleeds should be considered nonresponsive if the clinical situation meets the specified criteria 24 hours from the start of treatment. Conclusion: Although it is not intended to replace clinical judgment, this definition can guide the optimal course of treatment for patients with haemophilia and inhibitors.