Bleeding disorders in adolescents with heavy menstrual bleeding in a multicenter prospective US cohort

Zia, Ayesha; Jain, Shilpa; Kouides, Peter A.; Zhang, Song; Gao, Ang; Salas, Niavana; Lau, May; Wilson, Ellen E.; DeSimone, Nicole; Sarode, Ravindra

doi:10.3324/haematol.2019.225656

Cited by 44 publications

(41 citation statements)

References 35 publications

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“…Later age at evaluation for HMB could be related to a missed diagnosis before or accrual of hemostatic challenges with age prompting a referral to a tertiary care center resulting in full assessment for BD. Adolescents not presenting to the ED for evaluation and management had a higher chance of BD based on prior family history or severe bleeding symptoms and the caregivers were likely better prepared to manage HMB at the onset of menarche or seek care at specialized hemostasis clinics instead of utilizing ED 12 . Similarly, preparedness for HMB led to the early institution of treatments leading to less refractoriness.…”

Section: Discussionmentioning

confidence: 99%

“…[5][6][7][8][9][10][11] A recent study from Zia et al has, for the first time, prospectively followed a large cohort of adolescent girls with HMB to reveal the presence of an undiagnosed BD in nearly 33%. 12 Previously undiagnosed BD as a cause of HMB may remain elusive, especially if it is mild in severity, and HMB is the first or only presenting bleeding symptom. 13 Further, HMB in adolescents can be multifactorial; those with underlying BD can also experience anovulatory bleeding, delaying timely referral to a hematologist.…”

Section: Introductionmentioning

confidence: 99%

“…Underlying bleeding disorders (BD) are another common etiology with reported incidence rates ranging widely from 10% to 62%, and higher numbers reported from specialized hemostasis centers 5‐11 . A recent study from Zia et al has, for the first time, prospectively followed a large cohort of adolescent girls with HMB to reveal the presence of an undiagnosed BD in nearly 33% 12 …”

Section: Introductionmentioning

confidence: 99%

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Prospective evaluation of ISTH‐BAT as a predictor of bleeding disorder in adolescents presenting with heavy menstrual bleeding in a multidisciplinary hematology clinic

Jain

Zhang

Acosta³

et al. 2020

Journal of Thrombosis and Haemostasis

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Background: Heavy menstrual bleeding (HMB) can be the first manifestation of an undiagnosed bleeding disorder (BD). Identifying a BD can be challenging in the adolescent age group. The utility of bleeding assessment tools (BAT) remains elusive in this population. Aim: We evaluated the ability of the International Society on Thrombosis and Haemostasis-BAT (ISTH-BAT) in predicting a BD in adolescents referred for HMB to a multidisciplinary hematology clinic. Methods: Two hundred adolescents with HMB underwent a standardized evaluation for BD. The clinical characteristics, laboratory data, and bleeding scores (BS) assessed using ISTH-BAT were prospectively collected. Comparisons were made between patients based on the diagnosis of BD receiver operating characteristic (ROC) curve analyses of ISTH-BAT were performed to assess its value for predicting BD. Results: Overall, 33% (n = 67) of adolescents were diagnosed with a BD. The mean ISTH-BAT BS was higher in BD as compared to those without (4.1 versus 3.1, P < .0001), but the mean menorrhagia-specific scores did not differ (2.9 in both groups). The ISTH-BAT demonstrated a modest discriminative ability as a screening tool to identify BD in girls with HMB with an area under the curve (AUC) of 0.71. The ROC analysis demonstrated that with increasing BS, the sensitivity decreased, but the specificity increased. At BS = 3, sensitivity was 88%, specificity 31%, and accuracy 50%; at BS = 5, sensitivity was 37%, specificity 94%, and accuracy was 75%. There was no change thereafter. Conclusion: In adolescents with HMB, an ISTH-BAT BS of >4 instead of the established cutoff of >2 in children is highly specific in predicting a BD.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Prospective evaluation of ISTH‐BAT as a predictor of bleeding disorder in adolescents presenting with heavy menstrual bleeding in a multidisciplinary hematology clinic

Jain

Zhang

Acosta³

et al. 2020

Journal of Thrombosis and Haemostasis

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“…Intuitively, a proportion of adolescents with HMB due to anovulation should have an underlying bleeding disorder (BD), given the 11%-16% prevalence of von Willebrand disease (VWD) 3 and ~5% prevalence of platelet function disorders (PFD) in recent studies. 4 However, the diagnosis is usually not made until adulthood. 5 Multidisciplinary adolescent hematology clinics have helped closed the gap between HMB onset and BD diagnosis, 6 but only 5% of women with HMB present to a hematologist 7 or get screened for common BDs.…”

Section: Introductionmentioning

confidence: 99%

“…Heavy menstrual bleeding at the time of menarche is common and historically attributed to the immaturity of the hypothalamic‐pituitary‐ovarian axis. Intuitively, a proportion of adolescents with HMB due to anovulation should have an underlying bleeding disorder (BD), given the 11%‐16% prevalence of von Willebrand disease (VWD) 3 and ~5% prevalence of platelet function disorders (PFD) in recent studies 4 . However, the diagnosis is usually not made until adulthood 5 .…”

Section: Introductionmentioning

confidence: 99%

Standardizing care to manage bleeding disorders in adolescents with heavy menses—A joint project from the ISTH pediatric/neonatal and women's health SSCs

Zia

Kouides

Khodyakov

et al. 2020

Journal of Thrombosis and Haemostasis

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Background: Bleeding disorders (BD) are under-recognized in adolescents with heavy menstrual bleeding (HMB). Objectives: The lack of clinical guidelines and variable symptomatic management of HMB created the imperative to standardize HMB care to identify and manage BD in adolescents. Methods: We convened an international working group (WG), utilized the results of a literature review to define knowledge gaps in HMB care, and used the collective clinical experience of the WG to develop care considerations for adolescents with BD and HMB. We then solicited input on the appropriateness of HMB care considerations from expert stakeholders representing hematology, adolescent medicine, and obstetrics-gynecology. We conducted an expert panel online, using the ExpertLens platform. During a three-round online modified-Delphi process, the expert panel rated the appropriateness of 21 care considerations using a 9-point scale to designate care as appropriate (7-9), uncertain (4-6), or inappropriate (1-3) covering screening for

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Iron deficiency and fatigue among adolescents with bleeding disorders

et al. 2021

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Iron deficiency anemia is associated with heavy menstrual bleeding (HMB) and, by extension, a bleeding disorder (BD). It is unknown if iron deficiency without anemia is associated with a BD in adolescents. Moreover, the threshold of ferritin associated with fatigue in adolescents with HMB is unclear. In this multicenter study, we enrolled adolescents with HMB without BD. Participants underwent BD and anemia work-up in Young Women's Hematology Clinics and completed the Peds QL™ fatigue scale. BDs were defined as von Willebrand Disease, platelet function defect, clotting factor deficiencies, and hypermobility syndrome. Two hundred and fifty consecutive adolescents were enrolled, of whom 196 met eligibility criteria. Overall, 43% (95% confidence interval: 36%-50%) were diagnosed with BD. A total of 61% (n = 119) had serum ferritin levels < 15 ng/mL, 23.5% (n = 46) had iron deficiency only, and 37% (n = 73) had iron deficiency anemia. Low ferritin or ferritin dichoto-

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Bleeding disorders in adolescents with heavy menstrual bleeding in a multicenter prospective US cohort

Cited by 44 publications

References 35 publications

Prospective evaluation of ISTH‐BAT as a predictor of bleeding disorder in adolescents presenting with heavy menstrual bleeding in a multidisciplinary hematology clinic

Prospective evaluation of ISTH‐BAT as a predictor of bleeding disorder in adolescents presenting with heavy menstrual bleeding in a multidisciplinary hematology clinic

Standardizing care to manage bleeding disorders in adolescents with heavy menses—A joint project from the ISTH pediatric/neonatal and women's health SSCs

Iron deficiency and fatigue among adolescents with bleeding disorders

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