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DOI: 10.1055/s-0038-1670704
Long-Term Outcome after Joint Bleeds in Von Willebrand Disease Compared to Haemophilia A: A Post Hoc Analysis
Funding This research has been funded by unrestricted research grants from CLS Behring and Bayer.
Publication History
16 April 2018
08 August 2018
Publication Date:
01 October 2018 (online)


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 - HALNote
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.