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Sickle Cell Disease
(Sickle Cell Anemia)

Medical Author: William C. Shiel Jr., MD, FACP, FACR

What is sickle cell anemia?

Sickle cell anemia (sickle cell disease) is a disorder of the blood caused by an inherited abnormal hemoglobin (an oxygen-carrying protein within the red blood cells). The abnormal hemoglobin causes distorted (sickled) red blood cells. The sickled red blood cells are fragile and prone to rupture. When the number of red blood cells decreases from rupture (hemolysis), anemia is the result. This condition is referred to as sickle cell anemia. The irregular sickled cells can also block blood vessels causing tissue and organ damage and pain.

Picture of Sickle Cell Red Blood Cell

Sickle cell anemia is one of the most common inherited blood anemias. The disease primarily affects Africans and African Americans. It is estimated that in the United States, some 50,000 African Americans are afflicted with the most severe form of sickle cell anemia. Overall, current estimates are that one in 1,875 U.S. African American is affected with sickle cell anemia.



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Sickle Cell Disease (Sickle Cell Anemia)

What is anemia?

Anemia is a medical condition in which the red blood cell count or hemoglobin is less than normal. The normal level of hemoglobin is generally different in males and females. For men, anemia is typically defined as hemoglobin level of less than 13.5 gram/100ml and in women as hemoglobin of less than 12.0 gram/100ml. These definitions may vary slightly depending on the source and the laboratory reference used.

What causes anemia?

Any process that can disrupt the normal life span of a red blood cell may cause anemia. Normal life span of a red blood cell is typically around 120 days. Red blood cells are made in the bone marrow.

Anemia is caused essentially through two basic pathways. Anemia is either caused:

  1. by a decrease in production of red blood cell or hemoglobin, or
  2. by a loss or destruction of blood.

As more common classifications of anemia (low hemoglo...

Read the Anemia article »











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