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Abstract Thalassemia is an inherited blood disorder that can result in the abnormal formation of hemoglobin and is a genetic disorder inherited from a person‘s parents. Beta-thalassemia major is the most severe form of thalassemia, characterized by a severe microcytic, hypochromic iron deficiency. The external appearance is characterized by pallor, slight jaundice, feeding problems, diarrhea, irritability, recurrent bouts of fever, frontal bossing and other abnormalities of facies secondary to bone marrow expansion, and abdominal enlargement due to hepatosplenomegaly. Complications include hepatosplenomegaly and hyperslienism, iron overload, pulmonary hypertension, infections, abnormal bone structure, especially in the face and skull, growth failure, hypogonadism and impaired pancreatic β-cell function. SCA is an inherited autosomal recessive disease characterized by the presence of homozygous hemoglobin, Hb S. Sickling can be precipitated by environmental factors such as hypoxia, low pH, cold, and dehydration of the RBC, as well as adhesion molecules and cytokines associated with infections During hypoxic conditions, the red blood cell becomes sickled and the resulting change in structure restricts circulation causing obstruction of the blood flow within the capillaries and early destruction of the cell. |