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
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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