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

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http://hdl.handle.net/10138/340475

Lähdeviite

Maseide , R J , Berntorp , E , Nummi , V , Lassila , R , Tjonnfjord , G E & Holme , P A 2021 , ' Bleeding phenotype of patients with moderate haemophilia A and B assessed by thromboelastometry and thrombin generation ' , Haemophilia , vol. 27 , no. 5 , pp. 793-801 . https://doi.org/10.1111/hae.14355

Julkaisun nimi: Bleeding phenotype of patients with moderate haemophilia A and B assessed by thromboelastometry and thrombin generation
Tekijä: Maseide, Ragnhild J.; Berntorp, Erik; Nummi, Vuokko; Lassila, Riitta; Tjonnfjord, Geir E.; Holme, Pal A.
Tekijän organisaatio: HUS Comprehensive Cancer Center
Clinicum
Research Program in Systems Oncology
Department of Oncology
Hematologian yksikkö
Päiväys: 2021-09
Kieli: eng
Sivumäärä: 9
Kuuluu julkaisusarjaan: Haemophilia
ISSN: 1351-8216
DOI-tunniste: https://doi.org/10.1111/hae.14355
URI: http://hdl.handle.net/10138/340475
Tiivistelmä: 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 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.
Avainsanat: bleeding phenotype
joint score
moderate haemophilia A
moderate haemophilia B
thrombin generation
thromboelastometry
CALIBRATED AUTOMATED THROMBOGRAPHY
GLOBAL COAGULATION ASSAYS
ONE-STAGE
CONTACT FACTOR
COMMUNICATION
SSC
DIAGNOSIS
PROPOSAL
SCORE
3121 General medicine, internal medicine and other clinical medicine
Vertaisarvioitu: Kyllä
Tekijänoikeustiedot: cc_by_nc_nd
Pääsyrajoitteet: openAccess
Rinnakkaistallennettu versio: publishedVersion


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