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

Ragnhild J. Måseide, Erik Berntorp, Vuokko Nummi, Riitta Lassila, Geir E. Tjønnfjord, Pål A. Holme

Research output: Contribution to journalArticlepeer-review


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 (< median values) (P =.03). Factor VIII/factor IX activity (FVIII/FIX:C) did not align with the bleeding phenotype, but FIX:C ≤ 3 IU/dL was associated with lower peak thrombin (P =.03). Conclusion: TG differentiated patients with moderate haemophilia according to HJHS, annual factor consumption, and whether orthopaedic surgery had been performed. Thromboelastometry differentiated according to factor consumption only. Global coagulation assays may assist predicting the bleeding phenotype in moderate haemophilia.

Original languageEnglish
Pages (from-to)793-801
Issue number5
Early online date2021
Publication statusPublished - 2021

Subject classification (UKÄ)

  • Hematology

Free keywords

  • bleeding phenotype
  • joint score
  • moderate haemophilia A
  • moderate haemophilia B
  • thrombin generation
  • thromboelastometry


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