52 Chapter 2 with a severe bleeding phenotype, such as in patients with VWD type 3 46-48. Joint bleeds often lead to joint impairment and can hamper daily activities. Dysmenorrhea seems to be more frequent in women with heavy menstrual bleeding 29,30,32,39. Although some studies in the general population also describe an association between heavy menstrual bleeding and dysmenorrhea, no clear correlation has yet been identified 49-52. It has been hypothesized that heavy menstrual bleeding leads to retrograde bleeding, which is the reflux of menstrual blood out of the uterine cavity and may result in endometriosis with pelvic pain 53,54. Another explanation is that heavy menstrual bleeding leads to increased uterus contractions accompanied with more pain. Four studies focused specifically on other autosomal inherited bleeding disorders than VWD 23,32,33,42. It is difficult to draw clear conclusions with regard to the impact of these disorders on PROs, as the disorders evaluated were heterogeneous and patient numbers were small. Generally, similar HRQoL domains were affected compared to patients with VWD. In contrast to research in patients with autosomal inherited bleeding disorders, quality of life research in patients with hemophilia has been conducted for several decades. Generally, patients with hemophilia have a higher burden of disease compared to most other inherited bleeding disorders. Patients need regular prophylactic treatment with coagulation factor concentrates and may suffer from hemarthrosis and synovitis with joint destruction. The HRQoL of patients with hemophilia is especially affected in the domains of general health, physical functioning and pain 55-57. This review found that in patients with VWD similar domains were affected, however this was most likely influenced by different disease symptoms. Heavy menstrual bleeding was reported as a main influence on HRQoL, which is in contrast to patients with hemophilia as this predominantly affects males. In both diseases however, the severity of disease is strongly related to the HRQoL of patients. In patients with VWD type 3, the most severe type of VWD, HRQoL is comparable to that observed in severe hemophilia, specifically in the domains physical functioning, general health and physical component, although patients with hemophilia scored lower 25. Our study has several limitations. Firstly, a variety of PROMs are used in autosomal inherited bleeding disorders. This heterogeneity makes it difficult to assess, quantify and report PROs. Therefore, reported results and conclusions
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