Bleeding phenotype of patients with moderate haemophilia A and B assessed by thromboelastometry and thrombin generation
Autor: | Erik Berntorp, Geir E. Tjønnfjord, Riitta Lassila, Pål Andre Holme, Vuokko Nummi, Ragnhild Johanne Måseide |
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Přispěvatelé: | HUS Comprehensive Cancer Center, Clinicum, Research Program in Systems Oncology, Department of Oncology, Hematologian yksikkö |
Rok vydání: | 2021 |
Předmět: |
medicine.medical_specialty
thromboelastometry COMMUNICATION 030204 cardiovascular system & hematology DIAGNOSIS Hemophilia A Haemophilia Hemophilia B Gastroenterology ONE-STAGE 03 medical and health sciences Tissue factor PROPOSAL 0302 clinical medicine Thrombin Internal medicine SCORE joint score Humans Medicine CALIBRATED AUTOMATED THROMBOGRAPHY SSC Genetics (clinical) Whole blood Factor VIII Hematology business.industry General Medicine medicine.disease CONTACT FACTOR Thrombelastography 3. Good health Thromboelastometry Cross-Sectional Studies Phenotype bleeding phenotype Coagulation thrombin generation GLOBAL COAGULATION ASSAYS 3121 General medicine internal medicine and other clinical medicine Orthopedic surgery business moderate haemophilia B moderate haemophilia A 030215 immunology medicine.drug |
Zdroj: | Haemophilia. 27:793-801 |
ISSN: | 1365-2516 1351-8216 |
Popis: | 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. (Less) |
Databáze: | OpenAIRE |
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