Correlation of Platelet Receptor Expression in Glanzmann Thrombasthenia with Clinical Presentation and Haematological Parameters

Ahmad  Cheema; Manzar Bozdar; Hamid Saeed  Malik; Muhammad  Hussain; Rafia  Mahmood; Aysha  Khan; Ayesha  Khurshid; Syeda Samia  Shafaat

doi:10.51253/pafmj.v76i2.12125

Authors

Ahmad Cheema Department of Haematology, Armed Forces Institute of Pathology/National University of Medical Sciences (NUMS), Rawalpindi Pakistan
Manzar Bozdar Department of Haematology, Armed Forces Institute of Pathology/National University of Medical Sciences (NUMS), Rawalpindi Pakistan
Hamid Saeed Malik Department of Haematology, Armed Forces Institute of Pathology/National University of Medical Sciences (NUMS), Rawalpindi Pakistan
Muhammad Hussain Department of Immunology, Armed Forces Institute of Pathology/National University of Medical Sciences (NUMS), Rawalpindi Pakistan
Rafia Mahmood Department of Haematology, Armed Forces Institute of Pathology/National University of Medical Sciences (NUMS), Rawalpindi Pakistan
Aysha Khan Department of Haematology, Armed Forces Institute of Pathology/National University of Medical Sciences (NUMS), Rawalpindi Pakistan
Ayesha Khurshid Department of Haematology, Armed Forces Institute of Pathology/National University of Medical Sciences (NUMS), Rawalpindi Pakistan
Syeda Samia Shafaat Department of Haematology, Armed Forces Institute of Pathology/National University of Medical Sciences (NUMS), Rawalpindi Pakistan

DOI:

https://doi.org/10.51253/pafmj.v76i2.12125

Keywords:

Bleeding Assessment Tool, Flow Cytometry, Glanzmann Thrombasthenia

Abstract

Objective: To correlate platelet receptor expression with clinico-haematological parameters in patients of Glanzmann Thrombasthenia.

Study Design: Cross-sectional study.

Place and Duration of Study: Armed Forces Institute of Pathology/National University of Medical Sciences (NUMS), Rawalpindi Pakistan, from Jan 2023 to Jan 2024.

Methodology: Patients of Glanzmann thrombasthenia (GT) diagnosed by light transmission aggregometry were evaluated for the expression of CD 41 and CD 61 on platelet surface by flow cytometry. Data was assessed using BD FACS DIVA software. The correlation between the haematological parameters, clinical presentation based on bleeding assessment tool (BAT), and GT subtypes based on flow cytometric analysis was assessed.

Results: A total of 73 patients of GT were included in this study with mean age of 5.24±1.86 years. There were 31 males (42.47%) and 42 females (63.01%). Out of these patients, 46(63.01%) were classified as Type I (CD41/CD61 was absent or less than 5%). 21(28.77%), as Type II (5-25% CD41/CD61) and 06(8.22%) as Type III (CD41/CD61 greater than 25%). BAT score showed a significant, strong and negative correlation with platelet receptor expression (r=0.607, p<0.001) showing higher scores in Type I GT with median score of 9 (IQR 8-11) as compared to Type II with median score of 8 (IQR 7-9) and Type III with median score of 6 (IQR 5-7). Epistaxis was the most common clinical feature.

Conclusion: Type I Glanzmann Thrombasthenia (GT) was found to be the most common in our population. Bleeding Assessment Tool score had significant correlation with platelet receptor expression and GT ....

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