• Nazish Saqlain, Tooba Fateen, Hammad Tufail, and Naghmana Mazher.
• Dr. Nazish Saqlain, FCPS (Hematology), Department of Hematology and Transfusion Medicine, The Children's Hospital & Institute of Child Health, Lahore, Pakistan.
• Pak J Med Sci. 2022 Mar 1; 38 (4Part-II): 791-795.

ObjectivesTo 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.MethodsIt 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.ResultsOut 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*).ConclusionISTH-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.Copyright: © Pakistan Journal of Medical Sciences.

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