الفهرس 
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Abstract
β-thalassaemias are a group of hereditary human diseases caused by more
than 200 mutations of the human β-globin gene, leading to low or absent
production of adult β-globin and an excess of α-globin, causing ineffective
erythropoiesis and low or absent production of HbA (adult haemoglobin) The excess amount of α-globin induces the formation of α-globin aggregates
in erythroid progenitors. These aggregates precipitate and adhere to the membrane
causing cellular damage, massive apoptosis of erythroid progenitors in the bone
marrow, and only limited production of red blood cells Improvement of the standard care of homozygous patients, with almost
doubling of their life expectancy, unveiled a higher incidence of additional
complications which have not been previously recognized. In particular, profound
haemostatic changes have been observed, manifesting biochemical evidence of
hypercoagulable state, with an increased risk of developing venous and arterial
thrombosis, mainly in splenectomized patients with β-thalassemia intermedia Hypercoagulability in patients with thalassemia has been attributed to
several factors. It is often a combination of these factors that leads to
thromboembolic events One of these factors is chronic platelet activation and increased platelet
aggregation P-selectin is a member of the selectin family and is localized in the
membranes of the α-granules of platelets and the Weibel-Palade bodies of
endothelial cells. It is expressed on the cell surface upon activation and is the first point of
contact for adhesion of leukocytes to activated platelets present in thrombi or to
activated endothelial cells, where it enables leukocyte rolling initiating the
adhesion cascade A soluble form of P-selectin (sP-sel) has been detected in human plasma Despite P-selectin is present within endothelial cells and platelets, the majority of
sP-selectin come from platelets. It’s suggested that sP-selectin a more reliable
marker of in vivo platelet activation than membrane P-selectin The aim of this study is to evaluate plasma level of sP-selectin in pediatric
patients with β thalassemia (β-thalassemia major and β-Thalassemia intermedia) in
pediatric department in El-Menoufiya University hospital by ELIZA This study was carried out on 36 thalassemic patients, 25 thalassemia major
and 11 thalassemia intermedia, diagnosed as β-thalassemia by CBC and Hb
electrophoresis and 20 apparently healthy children were served as controls. They
were matched in age and sex and socioeconomic standard Regarding to our results: sP-selectin was significantly higher in cases than
controls (p<0.001) As regard the correlation between sP-selectin and platelet count in cases a
strong positive correlation was found (p <0.001) Concerning the correlation between sP-selectin and splenectomy, mean sPselectinin non-splenectomized thalassemic patients is (46.6±3ng/ml) and in
splenectomized thalassemic patients (61.6±2.4ng/ml). So, we found that there is a
highly significant correlation between sP-selectin level and splenectomy. We found a negative strong correlation between pretransfusion HB and sPselectin (p <0.001) As regard correlation between sP-selectin with frequency of blood
transfusion, no significant correlation was found (P>0.05) Concerning WBCs count: we found that there is a strong positive correlation between WBCs count and sP-selectin, As regard correlation between sP-selectin and ferritin level, there is no correlation (p >0.05) The gained data from our study revealed that sP-selectin, a marker of platelet activation, is elevated in β-thalassemia patients than controls particularly in splenectomized patients with strong positive correlation with platelet count and WBCs count.