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Abstract The β-Thalassemia syndromes are most common hereditary chronic hemolytic anemia due to impaired globin chain synthesis. This impairment leads to deficient hemoglobin resulting in hemolytic anemia. Sickle cell disease is a genetic disorder which causes red blood cells (RBCs) to acquire a sickle shape under conditions of hypoxia, resulting in anemia, cell adhesion, vaso-occlusion, severe pain, stroke and organ failure. ANG plays several roles in angiogenesis which is a crucial process in the pathogenesis of several inflammatory, autoimmune and malignant diseases. Endothelial damage and inflammation make a significant contribution to the pathophysiology of sickle cell disease (SCD) and the beta-β-Thalassemia syndromes. Angiogenesis is triggered by hypoxia and ANG secretion is enhanced by hypoxia. The aim of this study was to compare the serum ANG level as a marker of angiogenesis in patients with beta thalassemia and sickle cell disease, and to assess the possible risk factors related to ANG level in those patients. This study was performed on 72 patients; 32 Summary 149 with β-thalassemia major, 20 patients with β-thalassemia intermedia and 20 patients with Sickle cell disease. All patients are subjected to complete history and full examination. The following investigations were done: Complete blood count, Hb electrophoresis, serum ferritin, and serum ANG level. The study revealed the following results: The ANG level was significantly higher in patients with SCD compared to β-Thalassemia group and control with significant elevation in those with severe form of SCD (HbSS) compared with HbS/beta-thal. This study revealed that β-Thalassemia major group had significantly higher ANG level compared to controls specially β- Thalassemia intermedia with non significant difference in ANG level between splenectomized thalassemic patients and non splenectomized thalassemic patients (p=0.199). In our study, patients with β-Thalassemia had significant higher transfusion index (99 ±31.6 ml/kg/year) and transfusion frequency per year (12 ±2.8) compared to those with SCD with no significant correlation between blood transfusion index per year (ml ̷ kg ̸ year) and ANG level in SCD, β-Thalassemia major, β- Thalassemia intermedia with a highly significant negative Summary 150 correlation between ANG level and frequency of blood transfusion only in SCD(r=-0.731, p<0.001). In the current study, There was a significant inverse correlation between ANG level and the age of patients in β- Thalassemia major group and β-Thalassemia intermedia (p<0.001) (P=0.009) respectively. In this study, there was a significant negative correlation between ANG level and age of onset of disease in β-Thalassemia major group and in β-Thalassemia intermedia group (r=-0.414, p=0.019) (r=-0.486, p=0.30) but not in SCD. In this study, there was direct significant correlation between serum level of ANG and age of first transfusion in β-Thalassemia major(r=0.379 p=0.032). In the present study, there was significant inverse correlation between ANG level and BMI in SCD (r=-0.545, p=0.013). In this study there was significant inverse correlation between ANG level and ferritin in β-thalassemia intermedia group(r=-0.573, p=0.008). In our study, there was highly significant inverse correlation between ANG level and desferoxamine or deferiprone duration of therapy among β-Thalassemia intermedia(r=-0.830, r=-0.774) (p<.001) respectively while there was a highly significant inverse correlation between serum ANG and total duration of chelation in β- Summary 151 Thalassemia intermedia (p<0.001) and β-Thalassemia major (p=0.003) but no significant correlation in SCD (P=0.384). In this study there was a significant inverse correlation between serum ANG and hydroxyurea duration in SCD (p=0.017).. In our study, WBC count were direct correlated with ANG level in patient with SCD only while platelet count were correlated inversely with ANG level in patient with β-Thalassemia major only. |