Utility of the ISTH bleeding assessment tool (BAT) in diagnosis of Glanzmann Thrombasthenia patients

  • Nazish Saqlain The Children's Hospital Lahore
  • Tooba Fateen
  • Hammad Tufail
  • Naghmana Mazher
Keywords: Glanzmann Thrombasthenia, Platelet function disorders, Bleeding, Children, Questionnaire

Abstract

Objectives: To assess the utility of ISTH-BAT (International Society on Thrombosis and Hemostasis- Bleeding Assessment Tool) in the diagnosis of Glanzmann Thrombasthenia (GT) in comparison to controls.

Methods: It was a case-control study carried out at The Children’s Hospital, Lahore from January 2012 to May 2021. All patients from neonates to 18 years with a final diagnosis of GT were studied retrospectively. The clinical details were collected from hospital records and telephonically on ISTH-BAT questionnaire after taking informed consent. The same proforma was obtained from 75 healthy controls. Data was analyzed on SPSS version 26.

Results: Out of 427 patients with suspected platelet function disorders, 133 were diagnosed as GT. The mean age was 7.29±5 years. Male to female ratio was 1.1:1. Among cases, 76.6% were products of consanguineous marriage. Epistaxis was the commonest symptom with highest score (p value<0.001). Cutaneous and oral cavity bleeds were more severe and frequent in patients than controls (p value < 0.004). The median ISTH-BAT score among patients was nine while in control group was one. Sensitivity was 86.4%, specificity was 77.3%, positive predictive value was 0.87 and negative predictive value was 0.76. Area under the receiver operator curve was 0.78 (95% confidence interval 0.82–0.90, p<0.001*).

Conclusion: ISTH-BAT scores were significantly higher in GT patients than controls. So, we recommend the inclusion of ISTH-BAT in diagnostic evaluation of patients with suspected Glanzmann Thrombasthenia.

doi: https://doi.org/10.12669/pjms.38.4.5361

How to cite this:
Saqlain N, Fateen T, Tufail H, Mazher N. Utility of the ISTH bleeding assessment tool (BAT) in diagnosis of Glanzmann Thrombasthenia patients. Pak J Med Sci. 2022;38(4):791-795. doi: https://doi.org/10.12669/pjms.38.4.5361

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Published
2022-03-04
How to Cite
Saqlain, N., Fateen, T., Tufail, H., & Mazher, N. (2022). Utility of the ISTH bleeding assessment tool (BAT) in diagnosis of Glanzmann Thrombasthenia patients. Pakistan Journal of Medical Sciences, 38(4). https://doi.org/10.12669/pjms.38.4.5361