Summary The optimal management of menorrhagia among women with abnormal laboratory haemostasis is uncertain. In a crossover study, 116 women with menorrhagia [pictorial blood assessment chart (PBAC) score >100], negative gynaecological evaluation and abnormal laboratory haemostasis were randomly assigned to either intranasal desmopressin (IN‐DDAVP) or tranexamic acid (TA) therapy for two menstrual cycles. The subjects then crossed over to the second study drug for two additional cycles. Menstrual blood loss (MBL) was measured by PBAC scores at baseline and after each menstrual cycle. Quality of life (QOL) was assessed with four validated instruments. There was a statistically significant decrease in PBAC scores for both treatments. On average, the estimated decrease in the PBAC from baseline was −64·1 [95% confidence interval (CI) = −88·0, −40·3] for IN‐DDAVP and −105·7 (95% CI = −130·5, −81·0) for TA. The decrease in PBAC score was greater for TA than IN‐DDAVP (a difference of 41·6, P‐value = 0·0002, 95% CI = 19·6, 63·6). The test for treatment‐type effect was significant (P < 0·0001) suggesting a greater reduction in PBAC score with TA. Use of both IN‐DDAVP and TA improved QOL by all four instruments. We conclude that both medications reduced MBL and improved QOL among females with menorrhagia and abnormal laboratory haemostasis, but TA proved more effective.

SummaryInherited bleeding disorders are especially problematic for affected girls and women due to the monthly occurrence of menstrual periods and the effects on reproductive health. Although heavy menstrual bleeding (HMB) is the most common manifestation, females with inherited bleeding disorders (FBD) experience other bleeding symptoms throughout the lifespan that can lead to increased morbidity and impairment of daily activities.

While an estimated 13% of women with unexplained menorrhagia have von Willebrand disease (VWD), the frequency of other potential bleeding disorders has been uncertain. This study describes the relatively wide range of laboratory characteristics of women with unexplained menorrhagia and presents issues affecting diagnosis in this population. Women with pictorial blood assessment chart (PBAC) score > 100 were identified at six U.S. sites and asked to remain drug free for 10 days prior to testing. Blood was collected on one of the first four menstrual cycle days and tested at a central laboratory for procoagulant factors, VWD and fibrinolytic factors. Platelet function testing by PFA-100® (PFA) and platelet aggregation with ATP release (PAGG/ATPR) were performed locally using standardized methods. Among 232 subjects, a laboratory abnormality was found in 170 (73.3%), including 124 of 182 White (68.1%) and 34 of 37 Black (91.9%) subjects; 6.0% had VWD, 56.0% had abnormal PAGG/ATPR, 4.7% had a non-VWD coagulation defect (NVCD) and 6.5% had an abnormal PFA only. AGG/ATPR was reduced in 58.9% of subjects, with multiple agonists in 28.6%, a single agonist in 6.1% and ristocetin alone in 24.2%. Frequencies of PAGG/ATPR defects varied by study site and race; frequencies of VWD and NVCD were similar. Laboratory abnormalities of haemostasis, especially platelet function defects, were common among women with unexplained menorrhagia across multiple U.S. sites. To what degree these abnormalities are clinically significant requires further study.

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...

Introduction Health-related quality of life (HRQoL) is reduced among persons with haemophilia. Little is known about how HRQoL varies with complications of haemophilia such as inhibitors and joint disease. Estimates of preference-based HRQoL measures are needed to model the cost-effectiveness of prevention strategies. Aim We examined the characteristics of a national sample of persons with severe haemophilia A for associations with two preference-based measures of HRQoL. Methods We analysed utility weights converted from EuroQol 5 Dimensions (EQ-5D) and the Short Form 6 Dimensions (SF-6D) scores from 1859 males aged ≥14 years with severe haemophilia A treated at 135 US haemophilia treatment centres in 20052011. Bivariate and regression analyses examined age-group-specific associations of HRQoL with inhibitor status, overweight/obesity, number of bleeds, viral infections, indicators of liver and joint disease, and severe bleeding at the time of the first HRQoL measurement. Results Overall mean HRQoL utility weight values were 0.71 using the SF-6D and 0.78 using the EQ-5D. All studied patient characteristics except for overweight/obesity were significantly associated with HRQoL in bivariate analyses. In a multivariate analysis, only joint disease was significantly associated with utility weights from both HRQoL measures and across all age groups. After adjustment for joint disease and other variables, the presence of an inhibitor was not significantly associated with HRQoL scores from either of the standardized assessment tools. Conclusion Clinically significant complications of haemophilia, especially joint disease, are strongly associated with HRQoL and should be accounted for in studies of preference-based health utilities for people with haemophilia.

There are limited observational studies among children diagnosed with von Willebrand Disease (VWD). We analyzed differences in bleeding characteristics by sex and type of VWD using the largest reported surveillance database of children with VWD (n = 2712), ages 2 to 12 years old. We found that the mean ages of first bleed and diagnosis were lowest among children with type 3 VWD. It was even lower among boys than girls among all VWD types, with statistically significant difference among children with type 1 or type 3 VWD. Children with type 3 VWD also reported higher proportions of ever having a bleed compared to other VWD types, with statistically higher proportions of boys compared to girls reporting ever having a bleed with type 1 and type 2 VWD. A similar pattern was observed with the use of treatment product, showing higher usage among type 3 VWD, and among boys than girls with type 1 and type 2 VWD. While there were no differences in life quality or in well-being status by sex, children with type 3 VWD showed a greater need for mobility assistance compared to children with type 1 and type 2 VWD. In an adjusted analysis among children with type 1 VWD, boys showed a significant association of ever bleeding [hazard ratio 1.4; P-value <.001)] compared to girls. Understanding phenotypic bleeding characteristics, well-being status, treatment, and higher risk groups for bleeding among pre-adolescent children with VWD will aid physicians in efforts to educate families about bleeding symptoms.

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OBJECTIVE-To better understand the current evaluation of unexplained menorrhagia by obstetrician-gynecologists and the extent to which a bleeding disorder diagnosis is being considered in this population. STUDY DESIGN-A total of 1200 Fellows and Junior Fellows of the American College of Obstetricians and Gynecologists were invited to participate in a survey on blood disorders. Respondents completed a questionnaire regarding their patient population and their evaluation of patients with unexplained menorrhagia. RESULTS-The overall response rate was 42.4%. Eighty-two percent of respondents reported having seen patients with menorrhagia caused by a bleeding disorder. Seventy-seven percent of physicians reported they would be likely or very likely to consider a bleeding disorder as causing menorrhagia in adolescent patients; however, only 38.8% would consider bleeding disorders in reproductive age women. CONCLUSION-The current data demonstrate that obstetrician-gynecologists seem to have a relatively high awareness of bleeding disorders as a potential underlying cause of menorrhagia. Keywords bleeding disorder; menorrhagia; physician survey; practice patterns; von Willebrand disease Menorrhagia is a common clinical problem that affects approximately 30% of reproductiveage women. 1 Menorrhagia may result from various clinical conditions, including uterine fibroids, endometriosis, and cancer; however, a cause for menorrhagia is never identified in approximately 50% of cases. 2 Underlying bleeding disorders, including von Willebrand disease (VWD), other coagulation factor deficiencies, and platelet disorders, are prevalent among women with menorrhagia. VWD is caused by a quantitative or qualitative defect in