Congenital platelet disorders and health status-related quality of life

Maaike W. Blaauwgeers, Marieke J. H. A. Kruip, Erik A. M. Beckers, Michiel Coppens, Jeroen Eikenboom, Karin P. M. van Galen, Rienk Y. J. Tamminga, Rolf T. Urbanus, Roger E. G. Schutgens*, TiN Study Group

*Corresponding author for this work

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Background Patients with congenital blood platelet disorders (CPDs) demonstrate a predominantly mucocutaneous bleeding tendency. Repeated bleeds throughout life can have a significant impact on health status-related quality of life (HR-QoL), but few studies have investigated HR-QoL in patients with CPDs. Objectives To determine HR-QoL in patients with suspected or confirmed CPDs as compared with the general Dutch population and to assess the association between bleeding phenotype and HR-QoL. Methods Data were derived from the Thrombocytopathy in the Netherlands (TiN) study, a cross-sectional study of individuals suspected for a congenital platelet defect. TiN patients with an increased ISTH Bleeding Assessment Tool (ISTH-BAT) score (>3 in men and > 5 in women) were included for analysis. HR-QoL was assessed with the Short Form (SF)-36 survey. Bleeding symptoms were evaluated with the ISTH-BAT, resulting in a bleeding score. Results One hundred fifty-six patients were analyzed, of whom 126 (81%) were women. Sixty-two patients (40%) had a confirmed CPD. Compared to the general Dutch population, patients with a suspected or confirmed CPD reported decreased physical functioning, limitations in daily activities due to physical health problems, limitations in social activities, decreased energy levels and fatigue, pain, and lower general health status. HR-QoL was not correlated with the ISTH-BAT score and was similar in patients with a confirmed CPD and those in whom a CPD could not be diagnosed. Conclusion A bleeding tendency in patients with a suspected or confirmed CPD significantly impacts HR-QoL, independent of a confirmed explanatory diagnosis.

Original languageEnglish
Pages (from-to)100-105
Number of pages6
JournalResearch and practice in thrombosis and haemostasis
Issue number1
Publication statusPublished - Jan 2020


  • bleeding tendency
  • congenital blood platelet disorders
  • health status
  • quality of life
  • SF-36

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