Pediatric hereditary spherocytosis

Hereditary spherocytosis (HS) is a hereditary hemolytic disease caused by an abnormality of congenital erythrocyte membrane skeletal protein. Its main feature is that more small spherical red blood cells are seen in peripheral blood. Clinically it is characterized by anemia, jaundice, splenomegaly, increased erythrocytes in the blood, chronic anemia course and repeated acute episodes of hemolysis. It is now clear that HS is a genetic disease caused by abnormalities of the red blood cell membrane protein gene. The mechanism of hemolysis is mainly due to the defects in the structure of the red blood cell membrane that cause the red blood cells to become spherical. Ldquo; plasticity rdquo; reduces the occurrence of extravascular hemorrhage in the spleen when it is destroyed. It can occur at any age.