Sickle cell anemia is a recessive genetic disease that cannot be prevented or cured. This disease results in the body producing abnormal hemoglobin, the iron-rich and oxygen-carrying protein in red blood cells or erythrocytes. Due to the defective hemoglobin, the red blood cells are formed in sickle shapes rather than round discs. The abnormal, sickled red blood cells cause a wide range of complications and chronic symptoms that can affect every organ system in the body. The Journal of Occupational Medicine notes that sickle cell anemia can cause severe damage to organs including the spleen, an important immune system organ in the abdomen that also filters and stores blood cells.
Decreased Blood Flow
The symptoms and effects of sickle cell anemia are primarily due to the deformed red blood cells. The National Heart, Lung and Blood Institute explains that the sickled shape of red blood cells causes mechanical defects in the flow of blood through the blood vessels. Unlike normal, healthy spherical red blood cells that flow and bounce off each other easily, the sickled red blood cells clump and stick together. This causes blockages and clotting within the blood vessels and hinders the flow of vital blood, nutrients and oxygen to the organs and cells. The spleen and other organs of the body can become damaged and work more sluggishly due to this deficit in energy-producing oxygen and nutrients.
Splenic Crisis
If sickled red blood cells create clots or become trapped inside the spleen, the organ is unable to function correctly and can shrink, causing a splenic crisis. The National Heart, Lung and Blood Institute explains that if the spleen is shrunken it is unable to effectively filter blood. This increases the risk of infection in the body because this organ is essential in fighting germs. For this reason, infections such as meningitis, influenza and hepatitis are common in individuals with sickle cell anemia. In some cases, patients may require blood transfusions until the spleen is able to function adequately.
Splenic Pooling
Splenic pooling or sequestration is caused by sickled red blood cells accumulating in the spleen. This can cause an increased blood volume and enlargement of the spleen. This causes pain in the spleen and a sudden drop in hemoglobin, which is needed by the red blood cells to carry oxygen. The University of Maryland Medical Center warns that repeated episodes of splenic pooling can permanently damage and scar the spleen. In many cases, individuals with sickle cell anemia may be required to undergo surgical removal of the spleen. Most children with this disease do not have a functioning spleen before the age of 10 years due to extensive damage or removal of the organ.
