الفهرس 
Overview on ?- ThalassemiaOnly 14 pages are availabe for public view
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Abstract
Background: Beta-thalassemia syndromes are a group of hereditary blood disorders characterized by reduced or absent beta globin chain synthesis. A hypercoagulable state already exists in thalassaemic patients in early childhood. Aggregability of high levels of abnormal platelet-derived microparticle has been hypothesized to be responsible for the occurrence of thromboembolic events.
Aim of work: Our aim of this study was to assess the level of circulating platelet-derived microparticles (PDMPs) as a risk factor for hypercoagulable states in Egyptian children with β-thalassemia major.
Methods: Forty β-thalassemic children and thirty age- and sex-matched healthy subjects were enrolled as study group. We measured serum level of PDMPs and CBC for the study group. Assessment of systolic ventricular function and pulmonary artery pressure was done for all thalassemic cases using Doppler Echocardiographic study.
Results: Serum level of PDMPs was significantly elevated in thalassemic patients compared to healthy controls.Fifty percent of our cases had mild to moderate pulmonary hypertension. Splenectomized thalassemics had higher level of thrombocytosis and higher mean of pulmonary hypertension compared to non-splenectomized counterparts.
Conclusion: We suggest that increased level of microparticles may be implicated in vascular dysfunction and pulmonary hypertension risk observed in thalassemia patients. Their quantification could provide utility for early detection of cardiovascular abnormalities.
Keywords Beta-thalassemia; children; PDMPs; pulmonary hypertension.