Normal range of bleeding scores for the ISTH‐BAT: adult and pediatric data from the merging project

Abstract

Summary Bleeding Assessment Tools (BATs) have been developed to aid in the standardized evaluation of bleeding symptoms. The Vicenza Bleeding Questionnaire (BQ), published in 2005, established a common framework and scoring key that has undergone subsequent modification over the years, culminating in the publication of the ISTH-BAT in 2010. Understanding the normal range of bleeding scores is critical when assessing the utility of a BAT. Within the context of The Merging Project, a bioinformatics system was created to facilitate the merging of legacy data derived from four different (but all Vicenza-based) BATs; the MCMDM1-VWD BQ, the Condensed MCMDM-1VWD BQ, the Pediatric Bleeding Questionnaire and the ISTH-BAT. Data from 1040 normal adults and 328 children were included in the final analysis, which showed that the normal range is 0–3 for adult males, 0–5 for adult females and 0–2 in children for both males and females. Therefore, the cut-off for a positive or abnormal BS is ≥4 in adult males, ≥6 in adult females and ≥3 in children. This information can now be used to objectively assess bleeding symptoms as normal or abnormal in future studies.

Clinical and laboratory variability in a cohort of patients diagnosed with type 1 VWD in the United States

“…27 Furthermore, BS within each of the VWD groups varied; 24% of type 1 VWD subjects had BS in the normal range, which is a score of 0 to 3 for adult males, 0 to 5 for adult females, and 0 to 2 for all children ,18 years of age. 28 This pattern was present in both pediatric and adult subjects $18 years of age, suggesting that the variable bleeding phenotype seen in type 1 VWD is not solely a function of age and exposure to hemostatic challenges. There was no difference in BS between males and females in the type 1 cohort, but there was a significant difference between females and males in the historical VWD cohort (P , .001), with higher BS observed in adult female subjects.…”

“…It is possible that the BAT may be less sensitive in children with fewer hemostatic challenges, although different normal ranges are used in children. 28 In addition, our BAT was performed following diagnosis, such that some patients may have acquired higher scores due to a history of previous treatment of known VWD. Evaluation of BAT at time of diagnosis and following changes in BAT and VWF levels over time may be more predictive.…”

Application of whole‐exome sequencing to direct the specific functional testing and diagnosis of rare inherited bleeding disorders in patients from the Öresund Region, Scandinavia

“…We performed the International Society on Thrombosis and Haemostasis (ISTH) bleeding assessment tool (BAT) score (Elbatarny et al , 2014) in all patients referred to the Department of Haematology, Rigshospitalet (RH), Copenhagen University Hospital, Denmark, and the Coagulation Unit, Skaane University Hospital, Malmö, Sweden, between April 2013 and January 2016 on the basis of a suspected IBD. Patients without evidence of a coagulation factor deficiency [i.e.…”

Preoperative screening for bleeding disorders: A comprehensive laboratory assessment of clinical practice

“…The aPTT, PT, PFA, ELT and the ISTH‐BAT (using proposed BAT cut‐off values20) had high specificity but low sensitivity, indicating that these screening modalities cannot exclude mild coagulation factor, vWF or platelet function, and hyperfibrinolytic abnormalities, respectively (Table 5 and Supplementary Table S2). The PT performed best, with a sensitivity of 63% and NPV of 99%.…”

“…The area under the curve (AUC) with 95% confidence intervals (CI) was calculated. To demonstrate the diagnostic performance of the ISTH‐BAT when using previously proposed cut‐off values,20 we calculated diagnostic parameters with the cut‐offs of >5 for women and >3 for men.…”