Surveillance of female patients with inherited bleeding disorders in United States Haemophilia Treatment Centres

Byams, Vanessa R.; Kouides, Peter A.; Kulkarni, Roshini; Baker, Jeffrey R.; Brown, Deborah L; Gill, Joan Cox; Grant, Althea M.; James, Andra H.; Konkle, Barbara A.; Maahs, Jennifer; M, Dumas; McALISTER, S.; Nance, Danielle; Nugent, Diane J.; Philipp, Claire S.; Soucie, J. Michael; Stang, Ellen

doi:10.1111/j.1365-2516.2011.02558.x

Cited by 72 publications

(66 citation statements)

References 30 publications

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“…The incidence of HMB in the present study (50.0-53.3%) was concordant with the results of recent studies performed by Rae et al (29), Byams et al (30) and Kirtava et al (12) that reported incidence of 45.2, 51.7 and 44%, respectively. Regarding the study population, the women in the aforementioned studies were connected to hematology centers, as in the present study.…”

Section: Discussionsupporting

confidence: 83%

Heavy menstrual bleeding and health-associated quality of life in women with von Willebrand's disease

Govorov

Ekelund

Chaireti

et al. 2016

Experimental and Therapeutic Medicine

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Abstract. Women with the inherited bleeding disorder von Willebrand's disease (VWD) face gender-specific hemostatic challenges during menstruation. Heavy menstrual bleeding (HMB) can negatively affect their overall life activities and the health-associated quality of life. The purpose of the present study was to investigate whether women with VWD experienced HMB and an impaired health-associated quality of life. The study subjects were recruited from the Coagulation Unit of Karolinska University Hospital. Information was retrieved from various self-administered forms and medical records. Of the 30 women (18-52 years) that were included in the present study, 50% suffered from HMB, although the majority received treatment for HMB. In addition, almost all the included women perceived limitations in the overall life activities due to menstruation. The health-associated quality of life for women with HMB was significantly lower (P<0.10) with regards to 'bodily pain' compared with Swedish women of the general population. In conclusion, women with VWD experienced reduced health-associated quality of life as a result of HMB. Therefore, preventing limitations in overall life activities and improving their health-associated quality of life thorough counseling on menstrual bleeding is important for women with VWD. IntroductionVon Willebrand's disease (VWD) is the most common inherited bleeding disorder worldwide, with a prevalence varying within 0.1-1% of the general population depending on the method of diagnosis (1-4). The disorder is caused by a quantitative or qualitative deficiency in the von Willebrand factor (VWF). Since VWD is an autosomal inherited disorder, men and women are equally affected. However, more women experience hemostatic challenges through menstrual bleeding and childbirth (5).Heavy menstrual bleeding (HMB), which is defined as >80 ml per menstruation (6), is common in women with VWD. Previous studies have shown that 78-92% of women with VWD suffer from HMB, compared with a 10% prevalence of HMB in all Swedish women of reproductive age (7-9).HMB can be managed through a range of pharmacological treatments, such as contraceptive pills, levonorgestrel-releasing intrauterine systems (LNG-IUS), and tranexamic acid (TA) (10). For women with VWD, treatment with D-arginine vasopressin and VWF/factor VIII concentrates is also an option (11). Despite the availability of extensive pharmacological options, surgical treatment may be necessary (10). Kirtava et al (12) demonstrated that a higher proportion of women with VWD undergo hysterectomy and at a younger age compared with women in the general population.HMB affects numerous aspects of health-associated quality of life, both physical and mental. It can cause iron-deficiency anemia in severe cases, and there are psychological, social and employment consequences associated with HMB (13). Barr et al (14) demonstrated that women with VWD participate less in post-secondary education than the general population, and suggested that this may be due to iron defici...

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Section: Discussionsupporting

confidence: 83%

Heavy menstrual bleeding and health-associated quality of life in women with von Willebrand's disease

Govorov

Ekelund

Chaireti

et al. 2016

Experimental and Therapeutic Medicine

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“…Menorrhagia is the most common bleeding symptom in women of reproductive age with ARBDs [3][4][5][6][7][8], represents a very frequent cause of iron deficiency [1,3,6,9-12] and carries a high negative impact on the quality of life [2,7]. Furthermore, other frequent gynaecological problems, such as uterine fibroids, are more likely to be symptomatic because of the increased bleeding tendency.…”

Section: Introductionmentioning

confidence: 99%

“…Furthermore, other frequent gynaecological problems, such as uterine fibroids, are more likely to be symptomatic because of the increased bleeding tendency. In addition to menorrhagia, irregular menstrual cycles, breakthrough spotting, haemorrhagic ovarian cysts and endometriosis are other common gynaecological abnormalities reported in women with ARBDs [2,3,6]. With reference to the obstetric problems, bleeding may persist throughout pregnancy [12], especially when the haemostatic defect is severe.…”

Section: Introductionmentioning

confidence: 99%

Women with congenital factor VII deficiency: clinical phenotype and treatment options from two international studies

et al. 2016

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“…The combined incidence of hemophilia A and B has been reported to be 1 in 5000 live male births. Rarely, hemophilia A is also observed in female children 6 . 80% of patients have hemophilia A and 60-70% of these have severe hemophilia A and 32% have moderate and mild hemophilia A 7,8 .…”

Section: Discussionmentioning

confidence: 99%

A newborn with moderate hemophilia A with severe intracranial and extracranial hemorrhage: A case report

Kader¹,

Reis²,

Mutlu³

et al. 2017

Dicle Tıp Dergisi

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Intracranial hemorrhage among term newborns is a rare clinical condition with high morbidity and mortality. Although major bleeding is relatively uncommon, the incidence of intracranial hemorrhage in hemophilic children is higher during the first few days of life than at any other stage in childhood, which relates to the trauma of delivery. Here, we reported a newborn case diagnosed with moderate hemophilia A, without the presence of a positive family history of hemophilia and presenting with intracranial and extracranial hemorrhage and we aimed to emphasize that the early diagnosis and replacement therapy carries an essential importance. Keywords: newborn, hemophilia A, intracranial hemorrhage, extracranial hemorrhage. Ağır intrakranial ve ekstrakranial kanaması olan orta hemofili Özetİntrakranial kanamalar term yenidoğanlarda nadir görülen, morbidite ve mortalitesi yüksek bir klinik tablodur. Majör kanamalar nispeten nadir olmasına rağmen, hemofilik çocuklarda yaşamın ilk birkaç gününde doğum travmasına bağlı gelişebilecek intrakranial kanama insidansı, çocukluğun herhangi bir evresine göre daha yüksektir. Burada, ailesinde hemofili öyküsü bulunmayan, intrakranial ve ekstrakranial hemoraji ile başvurup orta derecede hemofili A tanısı konan bir yenidoğan olgusunu sunmayı ve erken teşhis ve replasman tedavisinin hayati bir öneme sahip olduğunu vurgulamayı amaçladık.

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Surveillance of female patients with inherited bleeding disorders in United States Haemophilia Treatment Centres

Cited by 72 publications

References 30 publications

Heavy menstrual bleeding and health-associated quality of life in women with von Willebrand's disease

Heavy menstrual bleeding and health-associated quality of life in women with von Willebrand's disease

Women with congenital factor VII deficiency: clinical phenotype and treatment options from two international studies

A newborn with moderate hemophilia A with severe intracranial and extracranial hemorrhage: A case report

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