Pulmonary hypertension in adult Egyptian patients with b-thalassemia major: correlation with natural anticoagulant levels

Background/aim Thalassemia constitutes a heterogeneous group of inherited anemia. In Egypt, 1000/1.5 million live births per year suffer from thalassemia. b-Thalassemia major (b-TM) is characterized by absent or reduced synthesis of b hemoglobin chains. The incidence and pathophysiology of pulmonary hypertension (PH) in patients with b-TM is not clear. The hypercoagulable state in thalassemia enhances the risk of thrombosis, such as pulmonary embolism. The aim of the present study was to investigate the prevalence of PH in adults with b-TM and investigate the role of protein C, protein S, and antithrombin III deficiencies in the pathogenesis of PH in adults with b-TM. Subjects and methods Forty patients with b-TM (group I) were selected, along with 20 age-matched and sex-matched healthy individuals as controls (group II). Patients were subjected to full medical history and complete clinical examination. Plasma protein C, protein S, and antithrombin III assays were measured using ELISA. Doppler echocardiography was used for estimation of systolic pulmonary artery pressure (sPAP). Results PH was diagnosed in 16 (40%) patients. Significantly higher levels of sPAP were found in poorly chelated and inadequately transfused patients. Significantly lower levels of protein C, protein S, and antithrombin III were found in patients with PH. There was significant negative correlation between protein C, protein S, and antithrombin III and sPAP. Conclusion Significant decrease in protein C, protein S, and antithrombin III was found in b-TM, especially in patients with PH, which might suggest the role of the hypercoagulable state in the pathogenesis of PH in b-TM.