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Utility of International Society on Thrombosis and Hemostasis Bleeding Assessment Tool (ISTH-BAT) in Patients with Inherited Bleeding Disorders: A Cross-Sectional Study from Southern India

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Abstract

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.

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References

  1. Rydz N, James PD (2012) The evolution and value of bleeding assessment tools. J Thromb Haemost 10:2223–2229

    Article  Google Scholar 

  2. Sramek A Sr, Eikenboom JC, Briet E, Vandenbroucke JP, Rosendaal FR (1995) Usefulness of patient interview in bleeding disorders. Arch Intern Med 155:1409–1415

    Article  CAS  Google Scholar 

  3. Rodeghiero F, Tosetto A, Castaman G (2007) How to estimate bleeding risk in mild bleeding disorders. J Thromb Haemost 5:157–166

    Article  Google Scholar 

  4. Rodeghiero F, Castaman G, Tosetto A, Batlle J, Baudo F, Cappelletti A, Casana P, De Bosch N, Eikenboom JC, Federici AB, Lethagen S, Linari S, Srivastava A (2005) The discriminant power of bleeding history for the diagnosis of type 1 von Willebrand disease: an international, multicenter study. J Thromb Haemost 3:2619–2626

    Article  CAS  Google Scholar 

  5. Tosetto A, Rodeghiero F, Castaman G, Goodeve A, Federici AB, Batlle J, Meyer D, Fressinaud E, Mazurier C, Goudemand J, Eikenboom J, Schneppenheim R, Budde U, Ingerslev J, Vorlova Z, Habart D, Holmberg L, Lethagen S, Pasi J, Hill F, Peake I (2006) A quantitative analysis of bleeding symptoms in type 1 von Willebrand disease: results from a multicenter European study (MCMDM-1 VWD). J Thromb Haemost 4:766–773

    Article  CAS  Google Scholar 

  6. Bowman M, Riddel J, Rand ML, Tosetto A, Silva M, James PD (2009) Evaluation of the diagnostic utility for von Willebrand disease of a pediatric bleeding questionnaire. J Thromb Haemost 7:1418–1421

    Article  CAS  Google Scholar 

  7. Sadler JE, Rodeghiero F (2005) Provisional criteria for the diagnosis of VWD type 1. J Thromb Haemost 3:775–777

    Article  CAS  Google Scholar 

  8. Boender J, Kruip MJHA, Leebeek FWG (2016) A diagnostic approach to mild bleeding disorders. J Thromb Haemost 14:1507–1516

    Article  CAS  Google Scholar 

  9. Rodeghiero F, Tosetto A, Abshire T, Arnold DM, Coller B, James P, Neunert C, Lillicrap D, ISTH/SSC joint VWF and Perinatal/Pediatric Hemostasis Subcommittees Working Group (2010) ISTH/SSC bleeding assessment tool: a standardized questionnaire and a proposal for a new bleeding score for inherited bleeding disorders. J Thromb Haemost 8:2063–2065

    Article  CAS  Google Scholar 

  10. Elbatarny M, Mollah S, Grabell J, Bae S, Deforest M, Tuttle A, Hopman W, Clark DS, Mauer AC, Bowman M, Riddel J, Christopherson PA, Montgomery RR, Zimmerman Program Investigators, Rand ML, Coller B, James PD (2014) Normal range of bleeding scores for the ISTH-BAT: adult and pediatric data from the merging project. Haemophilia 20:831–835

    Article  CAS  Google Scholar 

  11. Thomas W, Downes K, Desborough MJR (2020) Bleeding of unknown cause and unclassified bleeding disorders; diagnosis, pathophysiology and management. Haemophilia 26(6):946–957

    Article  Google Scholar 

  12. Perez Botero J, Warad DM, He R, Uhl CB, Tian S, Otteson GE, Barness RL, Olson MC, Gossman SC, Charlesworth JE, Nichols WL, Pruthi RK, Chen D (2017) Comprehensive platelet phenotypic laboratory testing and bleeding history scoring for diagnosis of suspected hereditary platelet disorders: a single-institution experience. Am J Clin Pathol 148:23–32

    Article  Google Scholar 

  13. Lowe GC, Lordkipanidze M, on behalf of the UK GAPP study group, Watson SP (2013) Utility of the ISTH bleeding assessment tool in predicting platelet defects in participants with suspected inherited platelet function disorders. J Thromb Haemost 11:1663–1668

    Article  CAS  Google Scholar 

  14. Adler M, Kaufmann J, Alberio L, Nagler M (2019) Diagnostic utility of the ISTH bleeding assessment tool in patients with suspected platelet function disorders. J Thromb Haemost 17(7):1104–1112

    Article  CAS  Google Scholar 

  15. Gebhart J, Hofer S, Panzer S, Quehenberger P, Sunder-Plassmann R, Hoermann G, Eigenbauer E, Haslacher H, Kepa S, Kyrle PA, Eichinger S, Knöbl P, Eischer L, Mannhalter C, Ay C, Pabinger I (2018) High proportion of patients with bleeding of unknown cause in persons with a mild-to-moderate bleeding tendency: results from the Vienna Bleeding Biobank (VIBB). Haemophilia 24(3):405–413

    Article  CAS  Google Scholar 

  16. Quiroga T, Goycoolea M, Panes O, Aranda E, Martínez C, Belmont S, Muñoz B, Zúñiga P, Pereira J, Mezzano D (2007) High prevalence of bleeders of unknown cause among patients with inherited mucocutaneous bleeding. A prospective study of 280 patients and 299 controls. Haematologica 92(3):357–365

    Article  Google Scholar 

  17. Tosetto A, Castaman G, Rodeghiero F (2013) Bleeders, bleeding rates, and bleeding score. J Thromb Haemost 11(Suppl 1):142–150

    Article  Google Scholar 

  18. Tosetto A, Castaman G, Plug I, Rodeghiero F, Eikenboom J (2011) Prospective evaluation of the clinical utility of quantitative bleeding severity assessment in patients referred for hemostatic evaluation. J Thromb Haemost 9:1143–1148

    Article  CAS  Google Scholar 

  19. Federici AB, Bucciarelli P, Castaman G, Mazzucconi MG, Morfini M, Rocino A, Schiavoni M, Peyvandi F, Rodeghiero F, Mannucci PM (2014) The bleeding score predicts clinical outcomes and replacement therapy in adults with von Willebrand disease. Blood 123:4037–4044

    Article  CAS  Google Scholar 

  20. Vries MJ, van der Meijden PE, Kuiper GJ, Nelemans PJ, Wetzels RJ, van Oerle RG, Lancé MD, Ten Cate H, Henskens YM (2018) Preoperative screening for bleeding disorders: a comprehensive laboratory assessment of clinical practice. Res Pract Thromb Haemost 2(4):767–777

    Article  Google Scholar 

  21. Fasulo MR, Biguzzi E, Abbattista M, Stufano F, Pagliari MT, Mancini I, Gorski MM, Cannavò A, Corgiolu M, Peyvandi F, Rosendaal FR (2018) The ISTH Bleeding Assessment Tool and the risk of future bleeding. J Thromb Haemost 16(1):125–130

    Article  CAS  Google Scholar 

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Acknowledgements

The authors acknowledge Mr. Sankar Perumal, Technical Officer, Department of Pathology, JIPMER for coagulation laboratory services, Mr. V. Balachandar, Research Assistant and Dr. Sitanshu Sekhar Kar, Additional Professor, Department of PSM, JIPMER for help in data analysis

Funding

This study was funded by JIPMER Intramural Grant for Ph.D. project.

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Authors and Affiliations

Authors

Contributions

PLA administered the questionnaire, performed the bench work and prepared the manuscript; DB co-guided the study and reviewed the manuscript; SCN reviewed the study periodically, provided critical inputs and reviewed the manuscript; RK conceptualized and guided the study, analyzed the results and edited the manuscript.

Corresponding author

Correspondence to Rakhee Kar.

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Conflict of interest

Authors declare that they have no conflict of interest.

Ethical Approval

This study was approved by the Institute Ethics Committee (JIP/IEC/2015/13/540 dated 23.04.15).

Ethical Standards

This study was conducted on patients who were enrolled in routine diagnostic care who were administered a questionnaire prior to their routine sampling. Healthy volunteers were administered the questionnaire. All were in accordance with the ethical standards of the Institute ethics committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed Consent

Informed written consent was obtained from the adult patients and from the parent or legal guardian for pediatric patients enrolled in the study according to Institute ethical guidelines.

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Preliminary data from the study were presented as posters in the ISTH SSC Meeting in Montpelier, France, 2016 and in Platelet Society meeting in Jesus College, Cambridge, 2019.

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Ambika, P.L., Basu, D., Nair, S.C. et al. Utility of International Society on Thrombosis and Hemostasis Bleeding Assessment Tool (ISTH-BAT) in Patients with Inherited Bleeding Disorders: A Cross-Sectional Study from Southern India. Indian J Hematol Blood Transfus 38, 122–131 (2022). https://doi.org/10.1007/s12288-021-01435-8

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