Bleeding phenotype of patients with moderate haemophilia A and B assessed by thromboelastometry and thrombin generation

Abstract

Introduction: Predicting the bleeding phenotype is crucial for the management of patients with moderate haemophilia. Global coagulation assays evaluate haemostasis more comprehensively than conventional methods. Aim: To explore global coagulation assays and the bleeding phenotype of patients with moderate haemophilia A (MHA) and B (MHB). Methods: The MoHem study is a cross-sectional, multicentre study covering Nordic patients with MHA and MHB. Thromboelastometry in whole blood and thrombin generation (TG) in platelet-poor plasma (1, 2.5 and 5 pM tissue factor (TF)) were compared with joint health (Haemophilia Joint Health Score (HJHS)) and treatment modality. Results: We report on 61 patients from Oslo and Helsinki: 24 MHA and 37 MHB. By TG (2.5 pM TF), patients who had been without replacement therapy during the previous 12 months depicted higher endogenous thrombin potential (P =.03). In contrast, those who had low ETP (< median) captured higher HJHS (P =.02). Patients who had undergone orthopaedic surgery generated least thrombin (P =.02). By thromboelastometry, those without the need of factor consumption had short clotting times, and quick times to maximum velocity