β-Thalassemia (β-thal) represents a major health problem worldwide and particularly in Egypt. Its prevention, compared to treatment, is cost-effective, possible and practical. In this study we evaluate a proposed paradigm for detection of the β-thal carrier state. The present study included 1627 children and adolescents of both sexes, presenting as outpatients to clinics of Ain-Shams University Hospitals, Cairo, Egypt, from November 1 2009 to June 30 2010. In the first phase, after performing a complete blood count (CBC), 280 microcytic hypochromic patients were selected. These cases were further analyzed by iron profile and high performance liquid chromatography (HPLC); in the second phase, hybridization detected 22 common β-globin mutations in 74.0% of the suspected cases. Thus, by HPLC, the Hb A2 level of >3.5% provided 100.0% sensitivity, 70.0% specificity, 75.0% positive predictive value (PPV), 100.0% negative predictive value (NPV) and accuracy of 70.0% to identify β-thal trait and at a cut-off of 4.0%, it provided 97.4% sensitivity, 72.7% specificity, 92.6% PPV, 88.8% NPV and a diagnostic accuracy of 92%. High performance liquid chromatography is a reliable and cost effective primary screening tool for β-thal trait at a Hb A2 level of ≥4.0%, while molecular testing is mandatory only for selected cases with borderline Hb A2 values between 3.5 and 4.0%.
Background: CD47 is an integrin associated protein which decreases with PRBCs (packed red blood cells) prolonged storage that possibly leads to less effective blood transfusion. Electrolyte imbalance occurs due to inactivation of Na+/K+ ATPase pump in low temperature which in turn leads to continuous increase of extracellular potassium, which is considered life threatening for patients with renal failure or those with massive transfusions. The objective of the present study is to evaluate the expression of CD47 by flow cytometry with other biochemical storage changes (pH, sodium and potassium ions) in CPD/SAGM packed non-leucoreduced Group (A) versus CPD/SAGM leucoreduced Group (B) red blood cell units at different storage times (on day 7, 14, 28 from date of red blood cells collection).Subjects and Methods: Nearly 450 ml of whole blood is obtained into quadruple blood bags and further subdivided into 20 non-leucoreduced units and 20 leucoreduced units. Non-leucoreduced and leucoreduced units are stored at 4 ± 2•C. Samples are taken from each packed red blood cell unit at days 7, 14 and 28 and tested for Na+ and K+ ions, pH and CD47 marker by flow cytometry.Results: Both leucoreduced and non-leucoreduced units show a highly significant decrease in Na+, pH and CD47 expression and a highly significant increase in K+ level. However, there was no statistically significant difference found between the 2 studied groups regarding Na+ and K+ levels, pH and CD47 expression on packed red cells except for Na+ on 28 th day which was slightly higher in leucoredced than non-leucoreduced units.
Conclusion:Red cell storage lesion is an inevitable process. Our study showed a highly statistically significant decrease in CD47 by flow cytometry in both leucoreduced and non-leucoreduced PRBCs along the storage period from day 7 to day 28.
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