Pediatric Definition - Alpha Thalassemia
Alpha Thalassemia
There are variation of alpha thalassemia which include deletion of one gene ( 3 alpha globin which appears asymptomatic ), deletion of two gene ( 2 alpha globin which appears asymptomatic or hypochromic blood smears with target and low mean corpuscular volume ) and three gene deletion ( 1 alpha globin) which present with microcytic anemia, hepatosplenomegaly, hypochromia, poikilocytosis and increased reticulocytosis known as Hgb disease. Four gene deletion may lead to intrauterine anemia and hydrop fetalis.
The common differential diagnosis of alpha thalassemia may include iron deficiency anemia, hemolytic anemia and beta thalassemia.
The investigations require may include Hgb electrophoresis, full blood count and blood smears.
The treatments may include transfusion, Hgb H folic acid, splenectomy due to hypersplenism and G6PD diet.
There are variation of alpha thalassemia which include deletion of one gene ( 3 alpha globin which appears asymptomatic ), deletion of two gene ( 2 alpha globin which appears asymptomatic or hypochromic blood smears with target and low mean corpuscular volume ) and three gene deletion ( 1 alpha globin) which present with microcytic anemia, hepatosplenomegaly, hypochromia, poikilocytosis and increased reticulocytosis known as Hgb disease. Four gene deletion may lead to intrauterine anemia and hydrop fetalis.
The common differential diagnosis of alpha thalassemia may include iron deficiency anemia, hemolytic anemia and beta thalassemia.
The investigations require may include Hgb electrophoresis, full blood count and blood smears.
The treatments may include transfusion, Hgb H folic acid, splenectomy due to hypersplenism and G6PD diet.
