Application of the ISTH bleeding score in hemophilia

Borhany, Munira; Fatima, Naveena; Abid, Madiha; Shamsi, Tahir; Othman, Maha

doi:10.1016/j.transci.2018.06.003

Cited by 17 publications

(13 citation statements)

References 15 publications

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“…Another disadvantage of BAT scores is that these scores increase in time and are not based on time intervals. Longitudinal BAT scores may overcome this …”

Section: Discussionsupporting

confidence: 88%

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Diagnostic work up of patients with increased bleeding tendency

et al. 2019

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Introduction The diagnostic trajectory of patients with increased bleeding tendency can be very costly and time‐consuming. In addition, previous studies have shown that half of these patients remain without final diagnosis despite all efforts. Aim This study aimed to improve insight into the current diagnostic process of these patients. Methods A total of 117 adult patients, referred to an academic hospital because of being suspected to have an increased bleeding tendency, were included. Different parameters were compared between patients receiving final diagnosis, patients without final diagnosis but a high Tosetto bleeding assessment tool (BAT) score (classified as bleeding of unknown cause, or BUC) and a control group consisting of patients without final diagnosis and a low BAT score. Results The BAT score was significantly higher in patients in the BUC group as compared to patients reaching final diagnosis (8.1 vs 4.9). Interestingly, the two subcategories most prevalently increased were surgery and post‐partum haemorrhage‐associated bleeding (surgery: 2.1 vs 1.1; post‐partum haemorrhage: 0.7 vs 0.0). Laboratory screening results were more often abnormal in patients reaching final diagnosis compared to patients remaining without diagnosis and a high BAT score (n = 32 (78%) vs n = 14 (46%), 95% CI 1.5‐12), especially concerning the PFA (=27 (66%) vs n = 10 (33%), 95% CI 1.4‐10) and von Willebrand factor activity levels (n = 11 (27%) vs n = 1 (3%), 95% CI 1.3‐91). Conclusion Isolated high bleeding score on surgical or post‐partum bleeding correlates with a lower chance of receiving final diagnosis. Withholding extensive haemostatic testing should be considered. Better screening and confirmative haemostatic assays are still needed.

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“…Another disadvantage of BAT scores is that these scores increase in time and are not based on time intervals. Longitudinal BAT scores may overcome this …”

Section: Discussionsupporting

confidence: 88%

“…Longitudinal BAT scores may overcome this. 28 In previous studies, measuring PFA was found to be an insensitive screenings assay, especially for patients with mild PFD's. 27,29 It was considered not very useful since the PFA cannot differentiate between different PFD's and vWD.…”

Section: Discussionmentioning

confidence: 97%

Diagnostic work up of patients with increased bleeding tendency

et al. 2019

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“…17 The ISTH-BAT has been investigated as a screening tool for bleeding disorders such as von Willebrand disease (VWD), qualitative platelet disorder (QPD), hemophilia, and rare bleeding disorders. [18][19][20][21][22][23][24][25][26][27][28][29][30] The clinical usefulness of ISTH-BAT in screening for an underlying BD in the adolescent girls presenting with HMB has not been investigated. With this study, we aimed to prospectively evaluate the ability of the ISTH-BAT in predicting the presence of a previously undiagnosed BD in a cohort of adolescent girls referred to specialty settings for HMB.…”

Section: Introductionmentioning

confidence: 99%

“…Abnormal ISTH‐BAT scores have been identified as ≥3 in children, ≥4 in adult males, and ≥6 in adult females 17 . The ISTH‐BAT has been investigated as a screening tool for bleeding disorders such as von Willebrand disease (VWD), qualitative platelet disorder (QPD), hemophilia, and rare bleeding disorders 18‐30 …”

Section: Introductionmentioning

confidence: 99%

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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“…The ISTH BAT has been validated in both children and adults in a variety of bleeding disorders including von Willebrand disease and the rare bleeding disorders 19‐22 . A recent study also showed that it was discriminatory in haemophilia as well 23 . In fact, the self‐assessed BAT has also been shown to be as effective in screening for abnormal bleeding as the healthcare professional administered BAT 24 .…”

Section: Alternative Approaches ‐ What Is Ideal and What Is Possible?mentioning

confidence: 99%

Diagnosis of haemophilia and other inherited bleeding disorders ‐ Is a new paradigm needed?

Srivastava

2020

Haemophilia

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The current paradigm for the diagnosis of haemophilia and other inherited bleeding disorders (IBDs) based on clinical assessment followed by screening tests and confirmation by assays of clotting factor levels or platelet functions is complex and cumbersome. These have been difficult to establish and standardize around the world for many reasons. Therefore, more than half of the expected number of people with these disorders in the world remain unidentified. A new approach is therefore needed. Use of validated bleeding assessment tools (BATs) offers an opportunity for standardized evaluation of clinically significant bleeding at the primary care level or even to be self‐assessed. Advances in genetic evaluation of these disorders through gene panels covering a wide range of IBDs based on next generation sequencing (NGS) technology enable the identification of the primary defect in the haemostasis system with high degree of accuracy. These methods can be centralized and made highly cost‐effective when done in large batches. The combination of high BAT score followed by NGS‐based genetic diagnosis could be the new paradigm for the primary diagnosis of IBDs. This will need to be followed by confirmation with functional haemostasis tests, as required. This approach will increase rates of detection of the known common disorders many folds and reduce the burden on these families towards accessing facilities for accurate diagnosis and appropriate treatment based on that.

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Application of the ISTH bleeding score in hemophilia

Cited by 17 publications

References 15 publications

Diagnostic work up of patients with increased bleeding tendency

Diagnostic work up of patients with increased bleeding tendency

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

Diagnosis of haemophilia and other inherited bleeding disorders ‐ Is a new paradigm needed?

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