title: Identifying non-responsive bleeding episodes in patients with haemophilia and inhibitors: a consensus definition.
abstract: 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 non-responsive to bypassing therapy with either Factor Eight Inhibitor Bypassing Activity (FEIBA; Baxter AG) or recombinant activated factor VII (rFVIIa; NovoSeven® , Novo Nordisk A/S) is essential to guide treatment decisions and optimize patient outcomes through early intervention. Although both bypassing agents are effective, differential responses to therapy necessitate multiple therapeutic options. This article provides a consensus definition for non-life-threatening joint and muscle bleeds that are non-responsive to bypassing agents. An international panel of seven physicians met in December 2008 to develop the consensus definition using a modified National Institutes of Health Consensus Development Conference method. The consequent definition of non-life-threatening bleeding episodes that are non-responsive to bypassing treatment provides a global picture of the condition of the patient during such an event. Identification of non-responsiveness 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 non-responsive if the clinical situation meets the specified criteria 24 h from the start of treatment. Although it is not intended to replace clinical judgment, this definition can guide the optimal course of treatment for patients with haemophilia and inhibitors.
authors.affiliation: Malmö University Hospital, Malmö, Sweden University Hospital of Wales, Cardiff, UK. erik.berntorp@med.lu.se
authors.forename: E
authors.initials: E
authors.lastname: Berntorp
authors.forename: P
authors.initials: P
authors.lastname: Collins
authors.forename: R
authors.initials: R
authors.lastname: D'Oiron
authors.forename: N
authors.initials: N
authors.lastname: Ewing
authors.forename: A
authors.initials: A
authors.lastname: Gringeri
authors.forename: C
authors.initials: C
authors.lastname: Négrier
authors.forename: G
authors.initials: G
authors.lastname: Young
mesh_terms: D019774:Blood Coagulation Factor Inhibitors; D001779:Blood Coagulation Factors / Q000627:therapeutic use*; D015942:Factor VIIa / Q000627:therapeutic use*; D006467:Hemophilia A / Q000150:complications* / Q000188:drug therapy*; D006470:Hemorrhage / Q000209:etiology* / Q000517:prevention & control; D006801:Humans; D007249:Inflammation / Q000150:complications; D009069:Movement Disorders / Q000209:etiology; D011994:Recombinant Proteins / Q000627:therapeutic use
chemical_list: D019774:Blood Coagulation Factor Inhibitors; D001779:Blood Coagulation Factors; D011994:Recombinant Proteins; C103587:recombinant FVIIa; C065655:anti-inhibitor coagulant complex; D015942:Factor VIIa