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Updated: May 13, 2025

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Correlation of Pulmonary Arterial Hypertension with Laboratory Parameters in Sickle Cell Disease- A Retrospective
Anindita Paul1, Priyanka Samal1, Kumar Gaurav Behera2
1Department of Clinical Hematology, Institute of Medical Sciences & Sum Hospital, Siksha 'O' Anusandhan Deemed to Be University, Bhubaneswar, Odisha 751003 India.
Abstract:
To study the prevalence of pulmonary arterial hypertension (PAH) in individuals with sickle cell disease (SCD) and its relationship with serological markers. Data of ninety-two SCD patients who visited the Department of Clinical Haematology between 2020 and 2021 was retrieved through the hospital record system. These patients underwent laboratory testing, including a clinical evaluation was done at baseline. They underwent Doppler echocardiography to determine the maximum tricuspid regurgitation jet velocity. Among 92 patients, 43 were men and 49 were female, with the mean age being 29.9 ± 8.8 years. Using Doppler echocardiography, their pulmonary artery systolic pressure was determined. Pulmonary arterial hypertension was found in 29 out of 92 patients (31.5%). The prevalence of PAH was 20.9% in males and 40.8% in women. A significant association was shown with females (p = 0.041.) There was a positive correlation with hemolytic markers, including reticulocyte count (r = 0.212, p < 0.05), lactate dehydrogenase (r = 0.322, p < 0.01 with tricuspid regurgitation velocity (TRvmax) (r = 0.322, p < 0.01) along with NT-ProBNP (r = 0.310, p < 0.01). The maximal tricuspid regurgitation velocity and haemoglobin concentration had a negative correlation. (r = -0.340, p < 0.01). In resource-limited settings, NT Pro BNP and transthoracic echocardiography can be used as screening tools for pulmonary hypertension in SCD.
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