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Abstract Background Heavy menstrual bleeding is an important health problem in women of reproductive age and is also one of the most common symptoms in women with bleeding disorders. Data about the frequency of rare bleeding disorders are limited, and population-based studies are lacking, so we aimed to determine the frequency of rare bleeding disorders among women presented with heavy menstrual periods that cannot be attributed to obvious problems. Methods Complete blood count and bleeding profiles include activated partial thromboplastin time, prothrombin time, factor VIII activity assay, ristocetin cofactor activity, von Willebrand antigen assay, platelet aggregation tests and other factor assays in 100 out of 300 females presented with unexplained heavy menstrual period, pictorial bleeding assessment chart (PBAC) > 100 as a screening tool for heavy menstrual periods, and or International Society of Thrombosis and Hemostasis-Bleeding Assessment Tool (ISTH-BAT) > 6. Results A total of 300 women with heavy menstrual periods without an obvious explained cause were included in our study. Among them, we found 100 (30%) females with a mean PBAC of 234 ± 147 and mean ISTH-BAT of 9 ± 5 denoting HMB may be due to underlying bleeding disorders. Among them, the most common diagnosis was VWD in 30 (30%). Other disorders were as follows: 28 (28%) cases with clotting factor deficiencies, 24 (24%) cases were found to have platelet dysfunction, and in 18% of our studied cases, we did not find a clear cause of their bleeding disorders (unknown). Conclusion Rare bleeding disorders are not uncommon and require comprehensive hemostatic evaluation as well as simple tools like PBAC and ISTH-BAT questionnaires for the identification of females presented with unexplained HMB.

Background: Heavy menstrual bleeding is an important health problem in women of reproductive age and is also one of the most common symptoms in women with bleeding disorders. Data about the frequency of rare bleeding disorders are limited, and population-based studies are lacking, so we aimed to determine the frequency of rare bleeding disorders among women presented with heavy menstrual periods that cannot be attributed to obvious problems. Methods: Complete blood count and bleeding profiles include activated partial thromboplastin time, prothrombin time, factor VIII activity assay, ristocetin cofactor activity, von Willebrand antigen assay, platelet aggregation tests and other factor assays in 100 out of 300 females presented with unexplained heavy menstrual period, pictorial bleeding assessment chart (PBAC) > 100 as a screening tool for heavy menstrual periods, and or International Society of Thrombosis and Hemostasis-Bleeding Assessment Tool (ISTH-BAT) > 6. Results: A total of 300 women with heavy menstrual periods without an obvious explained cause were included in our study. Among them, we found 100 (30%) females with a mean PBAC of 234 ± 147 and mean ISTH-BAT of 9 ± 5 denoting HMB may be due to underlying bleeding disorders. Among them, the most common diagnosis was VWD in 30 (30%). Other disorders were as follows: 28 (28%) cases with clotting factor deficiencies, 24 (24%) cases were found to have platelet dysfunction, and in 18% of our studied cases, we did not find a clear cause of their bleeding disorders (unknown). Conclusion: Rare bleeding disorders are not uncommon and require comprehensive hemostatic evaluation as well as simple tools like PBAC and ISTH-BAT questionnaires for the identification of females presented with unexplained HMB. [ABSTRACT FROM AUTHOR]

Background: The assessment of clinical history is crucial before referring a patient for further laboratory testing. Bleeding assessment tools (BAT) are developed to standardize clinical evaluation. A small number of patients with congenital fibrinogen deficiencies (CFDs) have been evaluated with these tools without definitive results. Aims: We compared the adequacy of the ISTH‐BAT and the European network of rare bleeding disorders bleeding score system (EN‐RBD‐BSS) to identify patients with CFDs. The correlation between the two BATs and fibrinogen levels and patient clinical grade severity was further analyzed. Methods: We included 100 Iranian patients with CFDs. Routine coagulation and fibrinogen‐specific tests (fibrinogen antigen [Fg:Ag] and activity [Fg:C]) were performed. The ISTH‐BAT and EN‐RBD‐BSS were used to assess the bleeding score (BS) of all patients. Results: The ISTH‐BAT and EN‐RBD‐BSS median (range) were 4 (0–16) and 2.21 (−1.49 to 6.71), with a statistically significant moderate correlation between the two systems (r =.597, P <.001). In patients with quantitative deficiencies (afibrinogenemia and hypofibrinogenemia), the correlation between Fg:C and the ISTH‐BAT was moderately negative (r = −.4, P <.001), while the correlation between Fg:C and the EN‐RBD‐BSS was weakly negative (r = −.38, P <.001). Overall, 70% and 72% of patients with fibrinogen deficiencies were correctly identified by both the ISTH‐BAT and EN‐RBD‐BSS, respectively. Conclusion: These results suggest that in addition to the ISTH‐BAT, the EN‐RBD‐BSS may also be useful in identifying CFD patients. We found a significant level of sensitivity for detecting fibrinogen deficiency in the two BATs, and bleeding severity classification correctly identified severity grades in almost two‐thirds of patients. [ABSTRACT FROM AUTHOR]

Background Glanzmann's thrombasthenia (GT) and Bernard-Soulier syndrome (BSS) are genetic bleeding syndromes characterized by defects in platelet aggregometry. Although these disorders are classified to be rare, their exact prevalence is still unknown; however, they are more frequent in communities where consanguineous marriages are common. Aim To study platelet surface glycoproteins expression using flow cytometry and to examine their correlation with bleeding severity using International Society of Thrombosis and Hemostasis–Bleeding Assessment Tools (ISTH-BAT) as bleeding score of choice. Patients and methods This case–control study included 51 patients with bleeding disorders recruited from the Department of Pediatric Hematology, Assiut University Hospital, in addition to 36 apparently healthy age- and sex-matched controls. All patients were tested for complete blood count (CBC), prothrombin time, partial thromboplastin time, platelet aggregation, and platelets surface glycoprotein analysis by flow cytometry. ISTH-BAT was used to register bleeding data for patients. Results GT and BSS had some similarities regarding the presentation and bleeding severity, but when CBC, platelet aggregation studies, and flow cytometric analysis were done, differentiation became much easier. GT patients showed a decrease in the expression of CD41 and CD61. Type I GT patients had more bleeding severity than type II and type III. BSS patients showed a decrease in expression of CD42b. There are correlations between the bleeding severity and CD41 in GT, and between the severity and CD42b in BSS. Conclusion Flow cytometric studies of platelet glycoproteins have great values in diagnosing BSS and GT, and further classifying GT cases into its three types. ISTH-BAT is a useful tool when dealing with platelet function disorders and has good sensitivity and ability to determine the severity. [ABSTRACT FROM AUTHOR]

The International Society on Thrombosis and Hemostasis bleeding assessment tool (ISTH-BAT) was developed to record bleeding symptoms and aid in patient diagnosis. This study was done to investigate the utility of ISTH-BAT in patients suspected to have inherited bleeding disorders. This cross-sectional study was conducted in a tertiary care hospital in Southern India over 3 and 1/2 years. A trained investigator administered the ISTH-BAT questionnaire to 432 patients undergoing evaluation for inherited bleeding disorder prior to routine coagulation screening and confirmatory tests and to 131 healthy volunteers as controls. Among patients, 42(9.7%) had primary hemostatic defect, 150(34.7%) had secondary hemostatic defects and 229(53%) had normal screening coagulogram with mean bleeding scores (BS) being 5.9, 6.9, and 4.2 respectively and the proportion of patients with abnormal BS being 69%, 88.7% and 59.4% respectively; the latter qualifying as unknown hemostatic defect. 11(2.5%) with acquired hemostatic defect on workup were excluded. The mean BS was 1.52 among healthy volunteers. Common bleeding patterns were epistaxis (73.8%), cutaneous bleeding (52.4%), hematuria (54.8%), menorrhagia (50%) in primary hemostatic defect; cutaneous bleeding (72%), muscle hematoma (58.7%), hemarthrosis (46.7%), menorrhagia (58.7%) in secondary hemostatic defects and epistaxis (45.9%), cutaneous bleeding (62.4%), menorrhagia (30.7%) in normal screening coagulogram group. Grade of bleeding was mostly 2 and sometimes 4 in primary, 2–4 in secondary and 1–2 in normal screening coagulogram group. ISTH-BAT is a valuable tool to record lifelong bleeding history. The pattern and score give clues regarding the nature and severity of the bleeding disorder. [ABSTRACT FROM AUTHOR]

Image, graphical abstract Receiver operating characteristic curves for the prediction of any diagnosis of a bleeding disorder by 2 different bleeding assessment tools (Vicenza BAT and ISTH BAT). : Bleeding assessment tools (BATs) have been developed to quantify bleeding severity. Their ability to predict for the diagnosis of a bleeding disorder has not been thoroughly investigated. : To evaluate the ability of the Vicenza BAT and the ISTH BAT to distinguish patients with an established bleeding disorder from those with bleeding of unknown cause (BUC). : Three-hundred fifty-nine patients (228 with BUC, 64%) from the Vienna Bleeding Biobank were assessed in this study. : The bleeding scores were similar in patients with an established diagnosis of a bleeding disorder compared to patients with BUC. Both BATs had a low sensitivity and specificity for the diagnosis of a bleeding disorder with areas under the receiver operating characteristic (ROC) curves of 0.53 (95% confidence interval 0.47–0.60) for the Vicenza BAT and 0.52 (0.46–0.59) for the ISTH BAT. In terms of specific diagnoses, both scores were most accurate in diagnosing von Willebrand disease (VWD, areas under the ROC curve; Vicenza BAT 0.67 (0.45–0.90); ISTH BAT 0.70 (0.50–0.90)). A separate evaluation of different bleeding symptoms in patients who had undergone surgery and tooth extraction revealed that postpartum bleeding and bleeding from small wounds was predictive for diagnosing a MBD in multivariable analysis. : The Vicenza- and the ISTH BAT have a low ability to distinguish patients with an established bleeding disorder from those with BUC. [ABSTRACT FROM AUTHOR]

Introduction: An increased bleeding tendency has been shown in female haemophilia carriers compared to healthy females. Bleeding assessment tools (BATs) have mainly been performed in western cultures. It is unclear how they perform in populations with different healthcare, health/wellness concepts and awareness, as well as family planning practices. Aim: To (a) describe and compare the bleeding symptoms in carriers with healthy females, particularly for bleeding after surgical abortion and intrauterine device (IUD) placement which are performed frequently for family planning in China; (b) quantify scores of International Society on Thrombosis and Haemostasis Bleeding Assessment Tool (ISTH‐BAT) and Chinese‐BAT (C‐BAT) developed to include surgical abortion and IUD placement as separate categories in Chinese haemophilia carriers; (c) correlate bleeding scores (BS) with factor levels. Methods: We conducted a multicentre, cross‐sectional study on obligate haemophilia carriers and healthy controls using ISTH‐BAT and C‐BAT. Results: We enrolled 125 haemophilia carriers and 106 controls. Carriers, compared to controls, had significantly higher median BS (3 vs 1 by both ISTH‐BAT and C‐BAT) and lower factor level (63.5 vs 101.8 IU/dL). Bleeding after surgical abortion and IUD placement was significantly associated with carrier status. Bleeding scores from neither ISTH‐BAT nor C‐BAT showed significant correlation with factor levels. Conclusion: Haemophilia carriers in China experienced abnormal bleeding. Unique to the Chinese carriers is significant bleeding after surgical abortion (3rd highest incidence of bleeding symptom) and IUD placement (4th highest). However, both ISTH‐BAT and C‐BAT exhibited no correlation between BS and factor levels in this population and neither could identify carriers with low factor level (of <50 IU/dL). [ABSTRACT FROM AUTHOR]

Introduction Standardized bleeding assessment tools (BATs), such as the International Society for Thrombosis and Hemostasis (ISTH)-BAT, are screening instruments used during the diagnostic workup of suspected bleeding disorders. A self-administered ISTH-BAT (self-BAT) would enhance screening and save time during an outpatient clinic visit. Aim This study was aimed to investigate the reliability and feasibility of the self-BAT. Methods The electronic self-BAT was created from the ISTH-BAT and paper-version of self-BAT and optimized by patients and physicians. Patients with a (suspected) congenital platelet defect (CPD), who had previously undergone physician-administered ISTH-BAT assessment, were invited to complete the self-BAT. Optimal self-BAT cut-off values to detect a bleeding tendency, as defined by the ISTH-BAT, were evaluated by receiver operator characteristic (ROC) curve analysis to reach a sensitivity ≥95%. Reliability was tested by assessing sensitivity, specificity, and intraclass correlation (ICC). Feasibility was evaluated on comprehension and length of self-BAT. Results Both versions of the BAT were completed by 156 patients. Optimal cut-off values for self-BAT to define a bleeding tendency were found to be identical to those of the ISTH-BAT. Normal/abnormal scores of the ISTH-BAT and self-BAT were agreed in 88.5% (138/156, 95% confidence interval [CI]: 0.83–0.93) of patients. The sensitivity and specificity of the self-BAT to detect a bleeding tendency were 96.9 and 48.1%, respectively. The ICC was 0.73. Self-BAT questions were graded by 96.8% (151/156) as “very easy,” “easy,” and “satisfactory” and questionnaire length as “exactly right” by 91% (142/156) of patients. Conclusion In patients with a (suspected) CPD, the self-BAT is sufficiently reliable and feasible to detect a bleeding tendency, which supports its use as a screening tool.

Introduction: The Hermansky–Pudlak syndrome (HPS) is an autosomal recessive rare disorder characterized by oculocutaneous albinism, bleeding diathesis, chronic granulomatous colitis and/or pulmonary fibrosis. HPS is the most common single‐gene disorder in Puerto Rico with a prevalence of 1:1,800 in the Northwest of the island. Risk of menorrhagia and post‐partum hemorrhage (PPH) in cases of women with HPS have been described in the medical literature, but data regarding comprehensive description of bleeding diathesis remains lacking. For this reason, we aim to identify bleeding events using the International Society on Thrombosis and Hemostasis Bleeding Assessment Tool (ISTH‐BAT), a standardized quantitative tool that translates the range of severity of bleeding symptoms into a cumulative bleeding score (BS). Objective: To use the ISTH‐BAT in HPS in order to describe bleeding symptoms and allow for comparison with other inherited bleeding disorders. Methods: Puerto Rican females and adult participants with HPS based on genetic linkage were enrolled. The ISTH‐BAT was administered and results were identified using descriptive statistical analysis. Results: Questionnaire answers of twelve women with HPS‐1 and HPS‐3 were evaluated. Participants' mean BS was HPS‐1 (11.4) and HPS‐3 (8.0) Participants with HPS‐1 and HPS‐3 reported abnormal bleeding events that presented during dental extractions, menorrhagia, surgical interventions, gastrointestinal, oral cavity and post‐partum. Patients with history of pulmonary fibrosis (PF) showed a higher mean bleeding score than those who had no history of PF. Conclusions: Female patients with HPS type 1 and 3 experienced abnormal bleeding events according to the ISTH‐BAT bleeding score. Bleeding medications were inconsistently used and varied independently from healthcare professionals. The benefits of this study were to understand the history of bleeding complications in patients with HPS type 1 and 3 using an international validated system. The results of this study will help design strategies to improve the care we provide to this population. [ABSTRACT FROM AUTHOR]

Background: Easy bruising is included as a major or minor criterion for the classification of multiple types of Ehlers-Danlos syndrome (EDS). Despite a longstanding recognition of the association between EDS and bleeding, we still lack a definitive understanding of the frequency, severity, and types of bleeding complications in patients with EDS., Objectives: To evaluate hemorrhagic symptoms using the International Society of Thrombosis and Haemostasis bleeding assessment tool (ISTH-BAT) in a cohort of patients with defined types of EDS., Methods: We utilized the ISTH-BAT to characterize hemorrhagic symptoms and their severity in a cohort of 52 patients with classical, classical-like, hypermobile, or vascular EDS and a matched group of 52 healthy control subjects., Results: The mean ISTH-BAT score was 0.1 for healthy subjects and 9.1 for patients with EDS (p < .0001). An abnormal ISTH-BAT score was observed in 32 of 52 (62%) patients with EDS and 0 of 52 healthy controls (p < .0001). The most frequent bleeding symptoms were bruising, muscle hematomas, menorrhagia, epistaxis, bleeding from the oral cavity, and bleeding after tooth extraction. Menorrhagia that was life-threatening or required surgery was reported in 7 of 52 (14%) patients with EDS., Conclusion: Patients with multiple types of EDS exhibit a wide range of bleeding symptoms ranging from mild to life-threatening episodes., Competing Interests: Declaration of competing interests There are no competing interests to disclose., (Copyright © 2023 International Society on Thrombosis and Haemostasis. Published by Elsevier Inc. All rights reserved.)

Introduction There has been increasing recognition in recent years that female carriers of haemophilia manifest abnormal bleeding; however, data on the use of bleeding assessment tools in this population are lacking. Aim Our objective was to validate the ISTH- BAT in haemophilia carriers to describe bleeding symptoms and allow for comparisons with factor levels and other patient groups. Methods This was a prospective, observational, cross-sectional study performed by members of Global Emerging HEmostasis Panel ( GEHEP). Unselected consecutive haemophilia carriers were recruited and a CRF and the ISTH- BAT were completed by study personnel. Results A total of 168 haemophilia carriers were enrolled: 155 haemophilia A and 13 haemophilia B. The mean age was 40 years (range: 20-82). Carriers had higher mean bleeding scores ( BS) compared with age-matched controls ( n = 46; 5.7 vs. 1.43; P < 0.0001) and Type 3 VWD OC ( n = 32; 3.0; P = 0.009), but lower BS compared with women with Type 1 VWD ( n = 83; 8.7; P < 0.0001). Fifteen carriers reported haemarthrosis, and of those six had normal FVIII/ FIX levels. There was a significant but weak negative correlation between BS and factor level (Spearman's r2 = −0.36, P < 0.001). Conclusion Our results show that haemophilia carriers experience abnormal bleeding, including haemarthrosis. Overall, BS in women with Type 1 VWD > haemophilia carriers > Type 3 VWD OC > controls. Understanding the performance of the ISTH- BAT in this population is a critical step in future research aimed at investigating the underlying pathophysiology of abnormal bleeding, with the ultimate goal of optimizing treatment. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Introduction  Standardized bleeding assessment tools (BATs), such as the International Society for Thrombosis and Hemostasis (ISTH)-BAT, are screening instruments used during the diagnostic workup of suspected bleeding disorders. A self-administered ISTH-BAT (self-BAT) would enhance screening and save time during an outpatient clinic visit. Aim  This study was aimed to investigate the reliability and feasibility of the self-BAT. Methods  The electronic self-BAT was created from the ISTH-BAT and paper-version of self-BAT and optimized by patients and physicians. Patients with a (suspected) congenital platelet defect (CPD), who had previously undergone physician-administered ISTH-BAT assessment, were invited to complete the self-BAT. Optimal self-BAT cut-off values to detect a bleeding tendency, as defined by the ISTH-BAT, were evaluated by receiver operator characteristic (ROC) curve analysis to reach a sensitivity ≥95%. Reliability was tested by assessing sensitivity, specificity, and intraclass correlation (ICC). Feasibility was evaluated on comprehension and length of self-BAT. Results  Both versions of the BAT were completed by 156 patients. Optimal cut-off values for self-BAT to define a bleeding tendency were found to be identical to those of the ISTH-BAT. Normal/abnormal scores of the ISTH-BAT and self-BAT were agreed in 88.5% (138/156, 95% confidence interval [CI]: 0.83-0.93) of patients. The sensitivity and specificity of the self-BAT to detect a bleeding tendency were 96.9 and 48.1%, respectively. The ICC was 0.73. Self-BAT questions were graded by 96.8% (151/156) as "very easy," "easy," and "satisfactory" and questionnaire length as "exactly right" by 91% (142/156) of patients. Conclusion  In patients with a (suspected) CPD, the self-BAT is sufficiently reliable and feasible to detect a bleeding tendency, which supports its use as a screening tool., Competing Interests: Conflict of Interest None declared.