ABSTRACT

A reduction in erythrocyte survival time is characteristic of a hemolytic anemia, and the underlying erythrocyte abnormality is often associated with an impairment of erythrocyte deformability. In patients with hemolytic anemia, however, loss of deformability of the pathologically altered erythrocyte may become an important determinant of its lifespan, the Cr-labeled erythrocyte survival time correlating with loss of erythrocyte filterability. Hereditary elliptocytosis is a congenital hemolytic anemia characterized by circulating elliptical erythrocytes. Irreversibly sickled cell have a shortened survival time in vivo and, within individual patients, their percentage in the blood correlates with the hemolytic rate. Rheological abnormalities of erythrocytes are commonly found in patients with a congenital hemolytic anemia. While an abnormality of membrane rheology may be characteristic of a particular disease, it is evident that any one rheological abnormality can be caused by a variety of protein defects.