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Congenital platelet disorders and health status-related quality of life.

ABSTRACT: 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.

SUBMITTER: Blaauwgeers MW

PROVIDER: S-EPMC6971322 | biostudies-literature | 2020 Jan

REPOSITORIES: biostudies-literature

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Congenital platelet disorders and health status-related quality of life.

Blaauwgeers Maaike W MW Kruip Marieke J H A MJHA Beckers Erik A M EAM Coppens Michiel M Eikenboom Jeroen J van Galen Karin P M KPM Tamminga Rienk Y J RYJ Urbanus Rolf T RT Schutgens Roger E G REG

Research and practice in thrombosis and haemostasis 20191211 1

<h4>Background</h4>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.<h4>Objectives</h4>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 ...[more]

PMID: 31989090

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Dataset Information

Congenital platelet disorders and health status-related quality of life.

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Congenital platelet disorders and health status-related quality of life.

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