Sickle Cell Anemia, a Molecular Disease

Abstract

The erythrocytes of certain individuals possess the capacity to undergo reversible changes in shape in response to changes in the partial pressure of oxygen. When the oxygen pressure is lowered, these cells change their forms from the normal biconcave disk to crescent, holly wreath, and other forms. This process is known as sickling. About 8 percent of American Negroes possess this characteristic; usually they exhibit no pathological consequences ascribable to it. These people are said to have sicklemia, or sickle cell trait. However, about 1 in 40 (4) of these individuals whose cells are capable of sickling suffer from a severe chronic anemia resulting from excessive destruction of their erythrocytes; the term sickle cell anemia is applied to their condition.