Women with inherited bleeding disorders are particularly disadvantaged as, in addition to suffering from general bleeding symptoms, they also have more opportunities to experience bleeding complications from regular haemostatic challenges during menstruation,
pregnancy and childbirth. Furthermore, such disorders pose important problems for affected women due to the associated reduction in quality of life caused by limitations in daily activities and work, and alteration of their reproductive life [1]. These latter problems include excessive menstrual bleeding or menorrhagia, miscarriage, bleeding complications during pregnancy and during/after delivery, and related complications such as acute or chronic anaemia. Consequently, the Ixazomib cell line management of these women is complicated due to considerable inter-individual variation. Moreover, reliable information on clinical management is relatively scarce with only a limited number of available long-term prospective studies of large cohorts providing evidence-based guidance relating AZD9668 nmr to diagnosis and treatment. Menorrhagia is defined as total blood loss exceeding 80 mL per cycle or
menses lasting longer than 7 days [2]. Recent estimates from the World Health Organisation are that 18 million women worldwide experience menorrhagia [2], although this estimate may represent a very heterogeneous group of women and it is important
to ensure correct diagnosis and treatment selleck chemicals options. Menorrhagia is a common presenting symptom among female patients with VWD and FIX deficiency, and carriers of haemophilia [1]. In carriers of haemophilia, the reported prevalence of menorrhagia has been estimated to be 10–57% [3]. From a historical perspective, it is interesting to note that the first patient identified with this disorder by Erik von Willebrand in 1926 eventually died of uncontrollable menstrual bleeding at age 13 years. Using the pictorial blood assessment chart (PBAC), women affected with VWD were shown to have significantly higher menstrual scores than their healthy peers. Published data point out that in type 1 VWD, it occurs in 79–93% of women [4,5], whereas, in women with type 2 and type 3 VWD, the prevalence ranges from 32% to 63% and 56–69% respectively [6,7]. Whether this difference is attributed to methodological limitations or has an underlying pathophysiological basis remains to be clarified. Following hysterectomy, women with VWD are also more likely to require blood transfusion and are less likely to be free of any bleeding complications than controls [8]. The management of menorrhagia requires the combined expertise of gynaecologists and haematologists, and needs to consider the age of the patient and whether or not the preservation of fertility is a requirement.
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