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Abstract
The β-thalassemias are hereditary disorders of hemoglobin (Hb) production characterized by defective Hb synthesis, which leads to shortened survival of red blood cells (RBCs) through hemolysis, and the premature death of RBC precursors within the bone marrow (i.e., ineffective erythropoiesis). These conditions result in chronic severe anemia and bone marrow expansion.
The aim of study is to investigate the state of hypercoagulability and platelet activation in patients with β-thalassemia.
The present study was carried on seventy five (75) diagnosed (newly and old) as β-thalassemiapatients based on Hemoglobin Electrophoresis. Protein C, Antithrombin III and D-Dimer was performed. Assessment of CD62 P, CD41, CD42 & CD61 expression by flow cytometer. B globin chain mutation detection by Strip assay method
Thrombosis was found in (7/75) patients (9.3%) from whole patients & found more in (group II patients) (6/25) (24%) than (group III patients) (1/25) (4%)and (group I patients) (0/25) (0%).
In this study, 48% and 50.7% of patients had lower-than-normal protein C and AT-III levels, respectively, and 10.7% had higher D dimer levels-than-normal as compared to healthy control.
The present study considered that the best cut-off values of D dimer, protein C, antithrombin-III and CD62P for discriminating thrombosis and non-thrombosis cases were >500 ng/ml, <60% , <67.5% and >50% respectively
In our study, thrombosis was present in 7 patients (9.3%). Homozygous (n=32), compound heterozygous (n=18), heterozygous (n=21) and wild type (n=4)
In conclusion, the present work reports the thrombotic risk assessment, hypercoagulability state and platelet activation from β thalassemia to highlight the factors responsible for hypercoagulation in these patients.