BETA-THALASSEMIA SYNDROMES, CLINICAL AND LABORATORY APPROACH

Authors : Emine TÜRKKAN, Su Gülsün BERRAK, Cengiz CANPOLAT

Pages : 194-200

View : 12 | Download : 9

Publication Date : 2016-12-03

Article Type : Review Paper

Abstract :The Beta insert ignore into journalissuearticles values(insert ignore into journalissuearticles values(3); thalassemia syndromes are a heterogeneous group of genetic disorders. The frequency of thalassemia is dependent on the ethnic origins of the patient population. Turkey is located in a geographic area of the world where thalassemia syndromes are common. The incidence rate of insert ignore into journalissuearticles values(3-thalassemia carriers was stated to be 2 per cent in Turkey. Clinical manifestations are diverse and range from asymptomatic hypochromia and microcytosis to profound anemia leading to death in early childhood if untreated. Individuals who are homozygous for the insert ignore into journalissuearticles values(3-thalassemia genes have severe, transfusion-dependent anemia and are said to have insert ignore into journalissuearticles values(3-thalassemia major. Thalassemia intermedia is a condition in which the degree of hemolysis is milder even though the patient may have a deficiency of both insert ignore into journalissuearticles values(3 genes. Therefore, thalassemia intermedia is essentially a descriptive term that refers to minimal or no need for transfusions. The presence of one normal gene in the heterozygotes usually leads to enough normal insert ignore into journalissuearticles values(3-globin chain synthesis so that the affected individuals are usually asymptomatic with only a mild anemia. This condition is referred to as insert ignore into journalissuearticles values(3-thalassemia minor or insert ignore into journalissuearticles values(3-thalassemia trait. In this report clinical and laboratory findings of 13- thalassemia syndromes in childhood are reviewed. Key Words: Beta thalassemia, Childhood, Anemia, Hypocromic microcytic anemia
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