ISTH/SSC bleeding assessment tool: a standardized questionnaire and a proposal for a new bleeding score for inherited bleeding disorders

Rodeghiero, Francesco; Tosetto, Alberto; Abshire, Thomas C.; Arnold, Donald M.; Coller, Barry S.; James, Paula; Neunert, Cindy; Lillicrap, David

doi:10.1111/j.1538-7836.2010.03975.x

Cited by 661 publications

(585 citation statements)

References 11 publications

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“…They were suffering from nosebleeds, haematomas and bleeding after surgery or tooth extraction. The score obtained by the BAT (ISTH Bleeding Assessment Tool, a questionnaire used to quantify bleeding symptoms) was higher in probands than in control population (7.0 ± 4.6 vs 0.8 ± 0.5, respectively), 23 regardless of gender. On analysing the haemostatic parameters, the probands showed mean VWF:Ag levels significantly (P = .002) lower than the heterozygotes from normal population (59.6 ± 5.4 and 128.0 ± 38.6 U/dL, respectively), but similar VWF:FVIIIB ratios ( Table 2).…”

Section: Haemorrhagic Symptoms In Type 2n Carriers and Type 2n Von mentioning

confidence: 99%

Type 2N von Willebrand disease: Characterization and diagnostic difficulties

et al. 2017

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Introduction An abnormal factor VIII (FVIII) binding capacity of von Willebrand factor (VWF) identifies type 2N von Willebrand disease (VWD). Type 2N VWD patients are identified by means of the VWF FVIII binding (VWF:FVIIIB) assay, and especially their VWF:FVIIIB/VWF:Ag ratio (VWF:FVIIIB ratio). Aim We report on our 15‐year experience of diagnosing type 2N VWD. Methods We have performed 2178 VWF:FVIIIB assays in bleeders and normal subjects. Results von Willebrand factor (VWF):FVIIIB was reduced in 682, but only 60 had low VWF:FVIIIB ratios (<0.74). Among nine patients who had a VWF:FVIIIB ratio below 0.3, four had normal VWF levels and were homozygotes for the p.R854Q mutation; the other five had low VWF levels due to a quantitative VWF mutation combined with p.R854Q. The VWF:FVIIIB ratio ranged between 0.3 and 0.73 in 51 subjects; 34 of them were heterozygotes for the p.R854Q mutation, while one carried the p.R760C. The heterozygotes for type 2N included subjects with or without bleeding symptoms, the former with significantly lower mean VWF levels than the latter. Among the 116 normal subjects tested, six were heterozygotes for the p.R854Q mutation (all asymptomatic). Conclusions The prevalence of type 2N in our VWD cohort was 2.5%, and 5.2% of the general population in Northeast Italy was found heterozygous for the p.R854Q mutation. It might be difficult to reveal a type 2N defect using routine tests alone, especially when it is combined with a quantitative VWF mutation. Accordingly, we always recommend VWF:FVIIIB assay in the diagnostic workup of VWD.

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Section: Haemorrhagic Symptoms In Type 2n Carriers and Type 2n Von mentioning

confidence: 99%

Type 2N von Willebrand disease: Characterization and diagnostic difficulties

et al. 2017

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“…He suffered from epistaxis and gum bleeding, and was prescribed oral tranexamic acid to control nose bleeding in the past. His BS was 4, according to the International Society on Thrombosis and Hemostasis bleeding assessment tool, 46 therefore prompting an evaluation for VWD. Based on VWF assays (FVIII:C 33 IU/dL, VWF:Ag 26 IU/dL, VWF:RCo 12 IU/dL, VWF:CBA 24 IU/dL, and RIPA 1.5 mg/mL), a tentative diagnosis of type 2M was made (VWF:RCo/VWF:Ag ratio 0.46 and VWF:CBA/VWF:Ag ratio 0.92); no relatives were available for study.…”

Section: Treatment Of Type 2 Vwd: a Case-based Approachmentioning

confidence: 99%

How I treat type 2 variant forms of von Willebrand disease

Tosetto

Castaman²

2015

Blood

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Type 2 von Willebrand disease (VWD) includes a wide range of qualitative abnormalities of von Willebrand factor structure and function resulting in a variable bleeding tendency. According to the current classification, 4 different subtypes can be identified, each with distinctive phenotypic and therapeutic characteristics. Current available laboratory methods allow a straightforward approach to VWD subtyping, and although the precise molecular characterization remains complex, it is not required for appropriate treatment of the vast majority of cases. Desmopressin can be useful only in a few type 2 cases compared with patients with actual quantitative deficiency (type 1), most often in variants with a nearly normal multimeric pattern (type 2M). However, since no laboratory test accurately predicts response to desmopressin, a trial test should always be performed in all type 2 VWD patients, with the exception of type 2B ones. Replacement therapy with plasma-derived von Willebrand factor-factor VIII concentrates represents the safe mainstay of treatment of all patients, particularly those not responding to desmopressin or requiring a sustained hemostatic correction because of major surgery or bleeding. A significant patient bleeding history correlates with increased bleeding risk and should be considered in tailoring the optimal antihemorrhagic prophylaxis in the individual patient.

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“…We participated in the ISTH‐BAT Repository maintained by the Rockefeller University Laboratory of Blood, Vascular Biology, and Informatics Core. The ISTH‐BAT consists of 14 bleeding items and the maximal score is 4 per item (latest version, updated July 19, 2011) 14. Information was administered to the Rockefeller website to retrieve bleeding scores 15…”

Section: Methodsmentioning

confidence: 99%

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

Vries

Meijden

Kuiper

et al. 2018

Research and Practice in Thrombosis and Haemostasis

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Essentials Patients with bleeding disorders are at risk of operative bleeding, but screening for these disorders is challenging.Patients with and without bleeding symptoms on a guideline‐based screening questionnaire were included and hemostatically phenotyped.The questionnaire could not differentiate between patients with and without hemostatic abnormalities.The discriminative power of the PT, aPTT, TT, Euglobulin lysis time, PFA, and the ISTH‐BAT was also limited. BackgroundPatients with mild bleeding disorders are at risk of perioperative bleeding, but screening for these disorders remains challenging.ObjectivesWe aimed to assess the prevalence of hemostatic abnormalities in patients with and without reported bleeding symptoms on a preoperative questionnaire, consisting of guideline‐proposed questions, and appraised the diagnostic value of several screening modalities for the identification of patients with hemostatic abnormalities.MethodsIn this observational study, 240 patients with and 95 patients without bleeding symptoms on the preoperative questionnaire were included. Patients with known bleeding disorders, antithrombotic drugs, thrombocytopenia, and anemia were excluded. Preoperatively, all patients underwent elaborate hemostatic testing. Hemostatic abnormalities were defined as coagulation, vWF, or fibrinolysis factor levels below reference range and platelet function defects. Screening modalities included the ISTH Bleeding Assessment Tool (ISTH‐BAT), PT, aPTT, TT, Euglobulin Lysis Time (ELT), and Platelet Function Analyser (PFA).ResultsIn 21 of 240 (8.8%) patients reporting bleeding symptoms, hemostatic abnormalities were found, including 7 reduced coagulation factor levels, 10 platelet function abnormalities, and 4 reduced vWF levels. In comparison, 10 of 95 (10.5%) patients not reporting bleeding symptoms had abnormalities. The ISTH‐BAT could not identify patients with abnormalities, while PT, aPTT, TT, ELT, and PFA had high specificity but low sensitivity to detect abnormalities.ConclusionsThe prevalence of hemostatic abnormalities in both patients with and without reported bleeding symptoms was 9%‐10%. This suggests that the guideline‐based questionnaire cannot differentiate between patients with and without abnormalities, while the discriminative power of the screening modalities is also limited.

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ISTH/SSC bleeding assessment tool: a standardized questionnaire and a proposal for a new bleeding score for inherited bleeding disorders

Cited by 661 publications

References 11 publications

Type 2N von Willebrand disease: Characterization and diagnostic difficulties

Type 2N von Willebrand disease: Characterization and diagnostic difficulties

How I treat type 2 variant forms of von Willebrand disease

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

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