title: Flow cytometric assessment of circulating platelet and erythrocytes microparticles in young thalassemia major patients: relation to pulmonary hypertension and aortic wall stiffness.
abstract: Heart disease is the leading cause of mortality and morbidity in β-thalassemia major (β-TM). Aggregability of abnormal red cells and membrane-derived microparticles (MPs) stemming from activated platelets and erythrocytes are responsible for thrombotic risk. We measured platelet and erythrocyte MPs (PMPs and ErMPs) in 60 young β-TM patients compared with 40 age- and sex-matched healthy controls and assessed their relation to clinicopathological characteristics and aortic elastic properties. Patients were studied stressing on transfusion history, splenectomy, thrombotic events, chelation therapy, hematological and coagulation profiles, flow cytometric measurement of PMPs (CD41b(+) ) and ErMPs (glycophorin A(+) ) as well as echocardiographic assessment of aortic elastic properties. Aortic stiffness index and pulmonary artery pressure were significantly higher, whereas aortic strain and distensibility were lower in TM patients than controls (P < 0.001). Both PMPs and ErMPs were significantly elevated in TM patients compared with controls, particularly patients with risk of pulmonary hypertension, history of thrombosis, splenectomy or serum ferritin >2500 μg/L (P < 0.001). Compliant patients on chelation therapy had lower MPs levels than non-compliant patients (P < 0.001). PMPs and ErMPs were positively correlated to markers of hemolysis, serum ferritin, D-dimer, vWF Ag, and aortic stiffness, whereas negatively correlated to hemoglobin level and aortic distensibility (P < 0.05). We suggest that increased MPs may be implicated in vascular dysfunction, pulmonary hypertension risk, and aortic wall stiffness observed in thalassemia patients. Their quantification could provide utility for early detection of cardiovascular abnormalities and monitoring the biological efficacy of chelation therapy.
authors.affiliation: Pediatrics Department, Faculty of Medicine, Ain Shams University, Cairo, Egypt. aagtfz@gmail.com
authors.forename: Azza A G
authors.initials: AA
authors.lastname: Tantawy
authors.forename: Amira A M
authors.initials: AA
authors.lastname: Adly
authors.forename: Eman A R
authors.initials: EA
authors.lastname: Ismail
authors.forename: Nevin M
authors.initials: NM
authors.lastname: Habeeb
mesh_terms: D000293:Adolescent; D001792:Blood Platelets / Q000378:metabolism* / Q000473:pathology; D055252:Cell-Derived Microparticles / Q000378:metabolism*; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D004912:Erythrocytes / Q000378:metabolism* / Q000473:pathology; D005260:Female; D005434:Flow Cytometry; D006461:Hemolysis; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood / Q000209:etiology / Q000503:physiopathology; D008297:Male; D015539:Platelet Activation; D012307:Risk Factors; D013927:Thrombosis / Q000097:blood / Q000209:etiology / Q000503:physiopathology; D059289:Vascular Stiffness; D017086:beta-Thalassemia / Q000097:blood / Q000150:complications / Q000503:physiopathology