Trends in clinical management of women with von Willebrand disease: a survey of 75 women enrolled in haemophilia treatment centres in the United States

Kirtava, A.; Crudder, Sally O.; Dilley, Anne; Lally, Cathy; Evatt, Bruce L.

doi:10.1046/j.1351-8216.2003.00832.x

Cited by 105 publications

(94 citation statements)

References 9 publications

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“…Supplementation of the screening tool with a low serum ferritin level yielded very similar results: 5% of the women (11/217) with hemostatic defects would have been missed; 93% of the women (143/154) with a hemostatic defect would have been stratified correctly for evaluation. As previously reported, 7 no additional benefit of adding the PFA-100 to the screening tool was found.…”

Section: Commentsupporting

confidence: 63%

“…Furthermore, few of these women are referred for hemostatic evaluation, despite the high prevalence of hemostatic abnormalities in this population 6 ; the average delay from onset of bleeding symptoms to diagnosis of a bleeding disorder has been reported to be 16 years. 7 Barriers to referral for hemostatic evaluation include difficulties gynecologists and primary care physicians have in determining whom to refer, lack of recognition by gynecologists and primary care physicians of menorrhagia as a symptom of a bleeding disorder, the size of the population with complaints of menorrhagia, and the lack of simple laboratory tests to screen for hemostatic abnormalities in this population. Given the under-recognition and delay in diagnosis of bleeding disorders and the potential for bleeding complications with surgery, childbirth or invasive procedures in women with menorrhagia, and unidentified bleeding disorders, a standardized screening tool to assist in the determination of which women to refer for hemostatic evaluation would be useful for the practicing gynecologist.…”

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confidence: 99%

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Evaluation of a screening tool for bleeding disorders in a US multisite cohort of women with menorrhagia

Philipp

Faiz

Heit

et al. 2011

American Journal of Obstetrics and Gynecology

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OBJECTIVE-The purpose of this study was to determine the usefulness of a simple screening tool for bleeding disorders in a multisite population of women with menorrhagia.STUDY DESIGN-Women with menorrhagia between the ages of 18 and 50 years from 6 geographically diverse US centers underwent hemostatic testing for bleeding disorders, complete blood cell count, and ferritin. A questionnaire that contained all elements of the 8-question screening tool was administered. Sensitivity of the screening tool, a screening tool with a pictorial blood assessment chart (PBAC) score of >185, and a screening tool with serum ferritin were calculated for hemostatic disorders.RESULTS-Two hundred and seventeen women who were identified with a PBAC score of ≥100 participated in the study. The sensitivity of screening tool was 89% for hemostatic defects, and sensitivity increased to 93% and 95% with a serum ferritin level of ≤20 ng/mL and PBAC score of >185, respectively.CONCLUSION-This study confirms the usefulness of a short screening tool for the stratification of women with menorrhagia for hemostatic evaluation. HHS Public AccessAuthor manuscript Am J Obstet Gynecol. Author manuscript; available in PMC 2015 October 08.Published in final edited form as: Am J Obstet Gynecol. 2011 March ; 204(3): 209.e1-209.e7. doi:10.1016/j.ajog.2010. Author Manuscript Author Manuscript Author Manuscript Author ManuscriptIt is estimated annually that approximately 5% of reproductive-age women seek medical attention for menorrhagia. 1,2 Underlying hemostatic abnormalities, which include decreased von Willebrand factor (VWF), platelet dysfunction, and decreased coagulation factors, are found commonly in women with menorrhagia. 1,3-5 Yet, most women with menorrhagia seek medical attention for their symptoms from gynecologists and primary care physicians, rather than from hematologists. Furthermore, few of these women are referred for hemostatic evaluation, despite the high prevalence of hemostatic abnormalities in this population 6 ; the average delay from onset of bleeding symptoms to diagnosis of a bleeding disorder has been reported to be 16 years. 7 Barriers to referral for hemostatic evaluation include difficulties gynecologists and primary care physicians have in determining whom to refer, lack of recognition by gynecologists and primary care physicians of menorrhagia as a symptom of a bleeding disorder, the size of the population with complaints of menorrhagia, and the lack of simple laboratory tests to screen for hemostatic abnormalities in this population. Given the under-recognition and delay in diagnosis of bleeding disorders and the potential for bleeding complications with surgery, childbirth or invasive procedures in women with menorrhagia, and unidentified bleeding disorders, a standardized screening tool to assist in the determination of which women to refer for hemostatic evaluation would be useful for the practicing gynecologist. Using data from women with menorrhagia at a single institution, a simple easy-to-administer sc...

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Section: Commentsupporting

confidence: 63%

mentioning

confidence: 99%

Evaluation of a screening tool for bleeding disorders in a US multisite cohort of women with menorrhagia

Philipp

Faiz

Heit

et al. 2011

American Journal of Obstetrics and Gynecology

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“…In a survey by the Centers for Disease Control and Prevention in the USA, 95% (71 of 75) of women receiving care in HTCs reported a strong positive opinion and satisfaction [160]. Similar positive findings were found among patients of the multidisciplinary clinic at the Katharine Dormandy Haemophilia Centre of the Royal Free Hospital in London [161].…”

Section: Platelet Function Analysismentioning

confidence: 64%

The obstetric and gynaecological management of women with inherited bleeding disorders – review with guidelines produced by a taskforce of UK Haemophilia Centre Doctors’ Organization

et al. 2006

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Summary. The gynaecological and obstetric management of women with inherited coagulation disorders requires close collaboration between obstetrician/ gynaecologists and haematologists. Ideally these women should be managed in a joint disciplinary clinic where expertise and facilities are available to provide comprehensive assessment of the bleeding disorder and a combined plan of management. The haematologist should arrange and interpret laboratory tests and make provision for appropriate replacement therapy. These guidelines have been provided for healthcare professionals for information and guidance and it is also intended that they are readily available for women with bleeding disorders.

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“…Even in the United States, it was reported that there was a median of 16 years (0-39 years) between the fi rst bleeding symptom and recognition of VWD in women. The average age at onset of bleeding symptoms was 6 years and at diagnosis of VWD was 23 years [18]. History of bleeding in surgery is important in bleeding disorders but this may be especially unhelpful in adolescents, because they might not yet have been exposed to such hemostatic challenges.…”

Section: Discussionmentioning

confidence: 99%

Screening Bleeding Disorders in Adolescents and Young Women with Menorrhagia

Kılıç¹,

Sarper²,

Zengin³

et al. 2013

tjh

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Abstract:Objective: Chronic menorrhagia causes anemia and impairment of life quality. In this study the aim was the screening of bleeding disorders in adolescents and young women with menorrhagia. Materials and Methods:The study was performed prospectively by pediatric hematologists. A form including demographic characteristics of the patients, bleedings other than menorrhagia, familial bleeding history, characteristics of the menorrhagia, and impairment of life quality due to menorrhagia was filled out by the researcher during a face-to-face interview with the patient. A pictorial blood assessment chart was also used for evaluation of blood loss. All patients underwent pelvic ultrasound sonography testing and women also received pelvic examination by gynecologists. Whole blood count, peripheral blood smear, blood group, serum transaminases, urea, creatinine, ferritin, PFA-100, PT, aPTT, INR, TT, fibrinogen, VWF:Ag, VWF:RCo, FVIII, and platelet aggregation assays were performed. Platelet aggregations were studied by lumiaggregometer. Results:Out of 75 patients enrolled, 60 patients completed the study. The mean age was 20.68±10.34 (range: 10-48) years and 65% (n=39) of the patients were younger than 18 years. In 18 (46%) of the adolescents, menorrhagia subsided spontaneously. In 20% (n=12) of the patients, a bleeding disorder was detected (1 case of type 3 von Willebrand disease, 2 patients with low VWF:Ag, 1 case of probable von Willebrand disease, 3 cases of Bernard-Soulier syndrome, 2 cases of Glanzmann thrombasthenia, 2 cases of immune thrombocytopenic purpura, 1 case of congenital factor VII deficiency). Conclusion:In patients with menorrhagia, at least complete blood count, peripheral smear, aPTT, PT, VWF:Ag, VWF:RCo, FVIII, and fibrinogen assays must be performed. When there is history of nose and gum bleeding, platelet function assay by lumiaggregometer must also be performed. In nearly 50% of adolescents, menorrhagia is dysfunctional and transient. Detailed coagulation assays can be postponed in adolescents if bleeding history other than menorrhagia and/or family history of bleeding and/or parental consanguinity is absent. All subjects with menorrhagia must consult with gynecologists and hematologists.

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Trends in clinical management of women with von Willebrand disease: a survey of 75 women enrolled in haemophilia treatment centres in the United States

Cited by 105 publications

References 9 publications

Evaluation of a screening tool for bleeding disorders in a US multisite cohort of women with menorrhagia

Evaluation of a screening tool for bleeding disorders in a US multisite cohort of women with menorrhagia

The obstetric and gynaecological management of women with inherited bleeding disorders – review with guidelines produced by a taskforce of UK Haemophilia Centre Doctors’ Organization

Screening Bleeding Disorders in Adolescents and Young Women with Menorrhagia

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