ADAMTS13 IN β-THALASSEMIC PATIENTS WITH OR WITHOUT HCV INFECTION AND CONTROLS

Abstract

he thalassemia syndromes are the most common hereditary chronic hemolytic anemia due to impaired globin chain synthesis. This impairment leads to deficient hemoglobin accumulation, resulting in hypochromic microcytic red cells, ineffective erythropoiesis and hemolytic anemia. It is inherited as autosomal recessive disorder. Patient with thalassemia are at high risk of acquiring a number of viral infections during multiple blood transfusions. Of these infections, hepatitis B, C and human immunodeficiency virus infections are extremely important. The aim of the current study was to measure ADAMTS13 level in patients with thalassemia with HCV infection and without HCV infection and controls of the same age and sex. The current study included 50 thalassemic patients attending the hematology clinic in Ain Shams Univerisity childrens' hospital, 25 patients with HCV and 25 without HCV. A well matched 25 controls regarding age (p=0.852), sex (p=1.00) and socioeconomic status (p=0.506) were included. A highly significant difference between thalassemic patients with and without HCV and control group regarding jaundice (higher in thalassemic with HCV p=0.0000) and family history of liver disease (higher in thalassemic with HCV p=0.000). No significant difference between the study groups regarding WBCs. Hemoglobin retics and ADAMTS13 were significantly lower in thalassemics with HCV (p≤0.001). While PT, PTT and serum ferritin were significantly higher in thalassemics with HCV (p≤0.001).Significant positive correlation was found between ADAMTS13 and platelet count (PLT), while negative correlations were found between ADAMTS13 and both serum ferritin and PTT. A significant higher amount of blood transfusion and