Congenital Hemolytic Jaundice (CHJ) (aka Hereditary Spherocytosis) (HS)

Congenital Hemolytic Jaundice (CHJ) (aka Hereditary Spherocytosis) (HS)

Dr. Kennedy
Congenital hemolytic jaundice, aka hereditary spherocytosis (HS), is a genetic disorder of the red blood cell membrane characterized by anemia, jaundice (yellowing of the skin and sclera) and splenomegaly (enlargement of the spleen). The red cells are smaller, rounder, and more fragile than normal. They have a spherical rather than the biconcave-disk shape of the normal red cell. These rotund red cells (spherocytes) are osmotically fragile and less flexible than normal red cells and tend to get trapped in narrow blood passages (the capillaries), particularly in the spleen, and there they break up (hemolyze) leading to hemolytic anemia. The clogging of the spleen with red cells almost invariably causes splenomegaly and the breakdown of these damaged red cells by the liver results in jaundice.

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