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Thromb Haemost 2018; 118(10): 1690-1700
DOI: 10.1055/s-0038-1670704
Coagulation and Fibrinolysis
Georg Thieme Verlag KG Stuttgart · New York

Long-Term Outcome after Joint Bleeds in Von Willebrand Disease Compared to Haemophilia A: A Post Hoc Analysis

Karin P. M. van Galen
1   Van Creveldkliniek, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands
,
Merel Timmer
1   Van Creveldkliniek, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands
,
Piet de Kleijn
1   Van Creveldkliniek, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands
2   Department of Rehabilitation, Physical Therapy Science and Sports, University Medical Centre Utrecht, Utrecht University, Utrecht, The Netherlands
,
Frank W. G. Leebeek
3   Department of Hematology, Erasmus University Medical Center, Erasmus University, Rotterdam, The Netherlands
,
Wouter Foppen
4   Department of Radiology, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands
,
Roger E. G. Schutgens
1   Van Creveldkliniek, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands
,
Jeroen Eikenboom
5   Department of Thrombosis and Hemostasis and Einthoven Laboratory for Vascular and Regenerative Medicine, Leiden University Medical Center, Leiden University, Leiden, The Netherlands
,
Karina Meijer
6   Department of Hematology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands
,
Karin Fijnvandraat
7   Department of Pediatric Hematology, Academisch Medisch Centrum, Emma Children's Hospital, Amsterdam, The Netherlands
,
Britta A. P. Laros-van Gorkom
8   Department of Hematology, Radboud University Medical Center, Radboud University, Nijmegen, The Netherlands
,
Jos W. Twisk
9   Department of Clinical Epidemiology and Biostatistics, VU University Medical Center, VU University, Amsterdam, The Netherlands
,
Eveline P. Mauser-Bunschoten
1   Van Creveldkliniek, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands
,
Kathelijn Fischer
10   Department of Epidemiology, Van Creveldkliniek and Julius Center, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands
,
on behalf of the WiN Studygroup› Author Affiliations
Funding This research has been funded by unrestricted research grants from CLS Behring and Bayer.
Further Information

Publication History

16 April 2018

08 August 2018

Publication Date:
01 October 2018 (online)

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Abstract

Long-term outcome after joint bleeds in von Willebrand disease (VWD) (von Willebrand factor activity ≤ 30 IU/dL) could differ from moderate or severe haemophilia A (HA) (factor VIII [FVIII] 1–5 IU/dL or FVIII < 1 IU/dL). We performed a post hoc analysis on Haemophilia Joint Health Score (HJHS, 0–124), X-ray Pettersson scores (PS, 0–13/joint) and the Haemophilia Activities List (HAL, 0–100), using multivariable regression to adjust for age (rate ratio [RR] or odds ratio [OR] [95% confidence interval]). We included 48 VWD (median age, 47 years, type 3 VWD, n = 19), 39 moderate HA (median, 39 years) and 59 severe HA patients (median, 25 years) with documented joint bleeds. VWD patients suffered repeated bleeding (lifetime > 5/joint) less often than moderate and severe HA patients (52% vs. 77% vs. 98%). HJHS and PS in VWD were similar to moderate HA (median HJHS 5 vs. 6, RR 0.9 [0.5–1.4] and PS > 3 of ≥ 1 joint OR 0.3 [0.1–1.4]), but better than in severe HA patients (median HJHS 5 vs. 9, RR 1.8 [1.1–2.9]; PS > 3 in any joint OR 0.1 [0.0–0.3]). Self-reported limitations in activities were comparable across VWD, moderate HA (HAL score < 95: 67% vs. 49%; OR 1.4 [0.5–3.6]) and young adults with severe HA (67% vs. 48%; OR 1.7 [0.7–4.4]). Despite fewer joint bleeds, joint outcome after joint bleeds was similar in VWD and moderate HA patients. Type 3 VWD patients had worst joint outcome, comparable to younger intensively treated severe HA patients. Limitations in activities occurred as often in VWD as in both moderate and severe HA.

Keywords

von Willebrand disease - joint bleed - arthropathy - haemophilia A - HJHS - Pettersson - HAL

Note

The authors presented part of the findings in this manuscript at the Scientific Conference on Bleeding Disorders of the European Hematology Association 2016, September 14–17, in Barcelona, Spain (oral presentation) and at the 58th Annual Congress of the American Society of Hematology 2016, December 1–6, in San Diego, United States.


Authors' Contributions

Karin P.M. van Galen: Performing research, writing the paper and analysed data. Merel Timmer and Piet de Kleijn: Performing research and writing the paper. Frank W.G. Leebeek: Supervising and analysing the data and writing the paper. W. Foppen: Performing research. Roger E.G. Schutgens, Jeroen Eikenboom, Karina Meijer, Karin Fijnvandraat and Britta A.P. Laros-van Gorkom: Made substantial contributions to the data analyses and writing of the paper. Jos W. Twisk: Designing data analysis plan and supervising writing the paper sections on statistical analysis and results. Evelien P. Mauser-Bunschoten and Kathelijn Fischer: Designing the research, supervising data analysis and writing the paper.


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