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.
Highlights
One of the most common hereditary bleeding disorders is Platelet function disorders (PFDs)
Out of 427 patients who presented with suspected platelet function disorders, 133 were diagnosed as Glanzmann Thrombasthenia on light transmission aggregometry (LTA)
History of bleeding in first degree relatives was present in 62.6% patients (p
Summary
One of the most common hereditary bleeding disorders is Platelet function disorders (PFDs). These patients can undergo bleeding after trauma or sometimes spontaneously in case of severity.[1]. In 1918, a rare mucocutaneous bleeding disorder was identified by Eduard Glanzmann, and it was named after the scientist as Glanzmann thrombasthenia (GT). The range of intensity of signs and symptoms of this disease is variable. It is diagnosed early in life due to symptoms like petechiae, purpura and easy bruising. Menorrhagia at childbearing age, epistaxis, bleeding from gums are quite common symptoms in this disease. Hematuria, bleeding from gastrointestinal tract and intracranial
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