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

Måseide, Ragnhild J. ; Berntorp, Erik LU ; Nummi, Vuokko LU ; Lassila, Riitta ; Tjønnfjord, Geir E. and Holme, Pål A. (2021) In Haemophilia 27(5). p.793-801
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.... (More)

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.

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author
; ; ; ; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
bleeding phenotype, joint score, moderate haemophilia A, moderate haemophilia B, thrombin generation, thromboelastometry
in
Haemophilia
volume
27
issue
5
pages
793 - 801
publisher
Wiley-Blackwell
external identifiers
  • pmid:34106506
  • scopus:85107594861
ISSN
1351-8216
DOI
10.1111/hae.14355
language
English
LU publication?
yes
id
01c9af24-5582-4680-ab2b-5e0d5bc4e75a
date added to LUP
2021-07-06 11:54:16
date last changed
2024-08-24 20:09:32
@article{01c9af24-5582-4680-ab2b-5e0d5bc4e75a,
  abstract     = {{<p>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 (&lt; 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 (&lt; 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.</p>}},
  author       = {{Måseide, Ragnhild J. and Berntorp, Erik and Nummi, Vuokko and Lassila, Riitta and Tjønnfjord, Geir E. and Holme, Pål A.}},
  issn         = {{1351-8216}},
  keywords     = {{bleeding phenotype; joint score; moderate haemophilia A; moderate haemophilia B; thrombin generation; thromboelastometry}},
  language     = {{eng}},
  number       = {{5}},
  pages        = {{793--801}},
  publisher    = {{Wiley-Blackwell}},
  series       = {{Haemophilia}},
  title        = {{Bleeding phenotype of patients with moderate haemophilia A and B assessed by thromboelastometry and thrombin generation}},
  url          = {{http://dx.doi.org/10.1111/hae.14355}},
  doi          = {{10.1111/hae.14355}},
  volume       = {{27}},
  year         = {{2021}},
}