Health-related quality of life is impaired in bleeding disorders of unknown cause: results from the Vienna Bleeding Biobank

Dino Mehic; Stephan Schwarz; Ihor Shulym; Cihan Ay; Ingrid Pabinger; Johanna Gebhart

doi:10.1016/j.rpth.2023.102176

Health-related quality of life is impaired in bleeding disorders of unknown cause: results from the Vienna Bleeding Biobank

Res Pract Thromb Haemost. 2023 Aug 22;7(6):102176. doi: 10.1016/j.rpth.2023.102176. eCollection 2023 Aug.

Authors

Dino Mehic^{1

2}, Stephan Schwarz¹, Ihor Shulym³, Cihan Ay¹, Ingrid Pabinger¹, Johanna Gebhart¹

Affiliations

¹ Department of Medicine I, Clinical Division of Haematology and Haemostaseology, Medical University of Vienna, Vienna, Austria.
² Centre of Physiology and Pharmacology, Institute of Vascular Biology and Thrombosis Research, Medical University of Vienna, Vienna, Austria.
³ IT-Systems and Communications, Medical University of Vienna, Vienna, Austria.

Abstract

Background: Bleeding disorder of unknown cause (BDUC) is a diagnosis of exclusion after extensive investigation of coagulation and platelet function and is commonly seen among patients with mild-to-moderate bleeding disorders. Despite increasing awareness among treating physicians, little is known about the health-related quality of life (HrQoL) in BDUC.

Objectives: To investigate HrQoL in patients with BDUC in comparison to the general population and patients diagnosed with other established bleeding disorders.

Methods: Patients with mild-to-moderate bleeding disorders from the Vienna Bleeding Biobank, a prospective cohort study, were contacted via mail and phone to complete the 36-Item Health Survey Questionnaire form.

Results: In total, 333/657 (50.7%) patients completed the 36-Item Health Survey Questionnaire. Patients with BDUC (n = 207, 62%) had significantly impaired HrQoL both in physical (47.8 vs 49.2) and mental health parameters (42.9 vs 51.0) compared to the general population (n = 2914, 56% females), which remained after adjustment for sex and age in multivariable linear regression. The impairment in HrQoL, compared to patients with von Willebrand disease, platelet function defects, or mild clotting factor deficiencies, did not prevail after adjustment for age and sex. In patients with BDUC, age and the presence of at least 1 comorbidity were associated with impaired physical health but not sex or bleeding severity. Of all analyzed bleeding symptoms, only joint bleeding was associated with impaired physical health and gastrointestinal bleeding with mental health in BDUC.

Conclusion: The impairments in HrQoL in patients with BDUC emphasize the burden of BDUC on mental and physical well-being, encouraging early recognition and better counseling of patients with BDUC.

Keywords: BDUC; mental health; physical health; platelet function defects; quality of life; von Willebrand diseases.