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

WiN Studygrp, Oct-2018, In : Thrombosis and Haemostasis. 118, 10, p. 1690-1700 11 p.

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  • Long-Term Outcome after Joint Bleeds in Von Willebrand Disease Compared to Haemophilia A

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DOI

  • WiN Studygrp

Long-term outcome after joint bleeds in vonWillebrand disease (VWD) (vonWillebrand factor activity 5/joint) less often thanmoderate 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 [0.53.6]) 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.

Original languageEnglish
Pages (from-to)1690-1700
Number of pages11
JournalThrombosis and Haemostasis
Volume118
Issue number10
Publication statusPublished - Oct-2018

    Keywords

  • von Willebrand disease, joint bleed, arthropathy, haemophilia A, HJHS, Pettersson, HAL, QUALITY-OF-LIFE, ACTIVITIES LIST, HEALTH SCORE, PROPHYLAXIS NETWORK, MODERATE HEMOPHILIA, ADULT PATIENTS, ARTHROPATHY, RELIABILITY, MANAGEMENT, ASSOCIATION

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