The bleeding score: Useful in predicting spontaneous bleeding events in adults with bleeding of unknown cause?

Relke, Nicole; Kuthiala, Shikha; Grabell, Julie; Hopman, Wilma M.; James, Paula

doi:10.1111/hae.13775

Cited by 17 publications

(26 citation statements)

References 10 publications

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“…Unlike patients with severe inherited bleeding disorders, it is likely these patients with mild‐moderate bleeding present later in life after recognition from multiple events. Recently, a cohort of patients with bleeding of unknown cause has been described in which there was no haemarthrosis or central nervous system bleeding, resembling our collection which also very rarely have such bleeding events . Patients with UBD are heavily treated; 61% of women for menorrhagia (excluding desmopressin) including 18% having hysterectomy, 17% patients for epistaxis and 23% patients have been transfused for bleeding either postoperatively or PPH (Table ).…”

Section: Discussionmentioning

confidence: 71%

“…21 (Table S1), which has recently been found in the Vienna Bleeding Biobank (VBB) to be associated with the diagnosis of a bleeding disorder and previously been described in association with bleeding from a range of causes , . [22][23][24][25] TxA and desmopressin and rarely platelet transfusion were successful in more than 90% of cases at reducing bleeding complications when given as haemostastic prophylaxis preprocedure; 16 of 69 procedures were high bleeding risk and no abnormal bleeding was observed ( 11 The variability we describe in management with platelets, TXA and desmopressin reflects the uncertainty of managing UBD given the lack of any clinical trials. Many patients, prior to diagnosis, experienced postoperative, postdental or PPH; 75%, 84% and 63%, respectively, for patients exposed to each risk.…”

Section: Discussionmentioning

confidence: 94%

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Characterization of a large cohort of patients with unclassified bleeding disorder; clinical features, management of haemostatic challenges and use of global haemostatic assessment with proposed recommendations for diagnosis and treatment

MacDonald

Wright

Beuche

et al. 2019

Int J Lab Hematology

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Introduction There is an unmet need to characterize the diagnosis and management of patients with an unclassified bleeding disorder (UBD). Methods Retrospective review of registered patients with UBD at our centre. Assessment including rotational thromboelastometry (ROTEM) and thrombin generation (TG) were used. Results A total of 124 patients were identified; 91% female. Mean age of presentation was 38.3 years. Mean bleeding score was 8.8 (standard deviation [SD] 3.8); 6.6 in men (SD 1.4) and 9.7 in women (SD 3.3), which was significantly different (P < .05). In women, after deduction of scores for menorrhagia and postpartum haemorrhage, the mean score was 6.4 which was not significantly different to the male score (P = .11). Twenty‐three percent of patients have been transfused, 61% women had treatment for menorrhagia and 17% for epistaxis. TxA and desmopressin were effective at preventing bleeding in 69 procedures and 13 deliveries. TG revealed 26% patients with a long lag time and 19% with a decreased endogenous thrombin potential but no diagnostic pattern was seen. ROTEM (NATEM) was unable to characterize patients; 9% had a prolonged clot time or maximum lysis. ThromboGenomics was normal in 45 tested patients. Conclusions We provide data which shows the bleeding score is biased towards gynaecological bleeding but which remains elevated even when the bleeding score is deducted. Tranexamic acid and desmopressin are effective as haemostatic prophylaxis but there is an urgent need for clinical trials. In conclusion, we describe the use of the bleeding score in these patients and phenotype, diagnosis (including ThromboGenomic testing) and management with practice recommendations.

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Section: Discussionmentioning

confidence: 71%

Section: Discussionmentioning

confidence: 94%

Characterization of a large cohort of patients with unclassified bleeding disorder; clinical features, management of haemostatic challenges and use of global haemostatic assessment with proposed recommendations for diagnosis and treatment

MacDonald

Wright

Beuche

et al. 2019

Int J Lab Hematology

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“…Our diagnostic process led to a final diagnosis in 58% of patients referred, which is in line with previous reports . Current laboratory assays have proven to be useful in detecting coagulation disorders.…”

Section: Discussionmentioning

confidence: 99%

“…Our diagnostic process led to a final diagnosis in 58% of patients referred, which is in line with previous reports. [6][7][8][9][10][11][12][13] Recently, several studies described the value of next-generation sequencing. 5,[32][33][34] In our clinic, whole exome sequencing (WES) was recently incorporated in the diagnostic trajectory of patients without a definite diagnosis but with a high bleeding score (cut-off BAT score ≥ 10).…”

Section: Discussionmentioning

confidence: 99%

“…4,5 Previous studies indicated that 28%-42% of patients referred for a bleeding tendency to a tertiary haematological clinic received a final diagnosis. [6][7][8][9][10][11][12][13] However, a significant group of patients remained without diagnosis. This may be due to a lack of sensitive and specific assays that adequately determine pathological alterations in the haemostatic balance, or due to a lack of specific bleeding assessment tools.…”

Section: Introductionmentioning

confidence: 99%

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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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Risk factors for future bleeding in patients with mild bleeding disorders: longitudinal data from the Vienna Bleeding Biobank

Mehic

Neubauer

Janig

et al. 2023

Journal of Thrombosis and Haemostasis

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The bleeding score: Useful in predicting spontaneous bleeding events in adults with bleeding of unknown cause?

Cited by 17 publications

References 10 publications

Characterization of a large cohort of patients with unclassified bleeding disorder; clinical features, management of haemostatic challenges and use of global haemostatic assessment with proposed recommendations for diagnosis and treatment

Characterization of a large cohort of patients with unclassified bleeding disorder; clinical features, management of haemostatic challenges and use of global haemostatic assessment with proposed recommendations for diagnosis and treatment

Diagnostic work up of patients with increased bleeding tendency

Risk factors for future bleeding in patients with mild bleeding disorders: longitudinal data from the Vienna Bleeding Biobank

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