The spleen is an organ in the body that filters the blood by removing damaged blood cells and bacteria.
In sickle cell disease, the spleen removes sickle-shaped red blood cells from the blood. This can result in the removal of too many red blood cells. When too many red blood cells are removed, a condition (anemia) results that causes symptoms such as pale skin and weakness.
Sickled red blood cells may also damage parts of the spleen, preventing it from working properly. When the spleen doesn't work properly, people with sickle cell disease are more likely to have serious, life-threatening infections with certain types of bacteria. In older children and adults, the spleen often disappears because of years of damage from sickled cells.
WebMD Medical Reference from Healthwise