Studies on the Destruction of Red Blood Cells

Abstract

The chief clinical features of sickle-cell anemia are chronic hemolytic anemia and episodic painful crises; the underlying defect is the presence of S hemoglobin* in the red blood cells from patients with this disease. The deoxygenation of S hemoglobin leads to the formation of irregular elongated molecular masses—tactoids—which give to the red cells the distorted shape from which the disease derives its name.^1,5The manner in which the sickling phenomenon accounts for the physiological and pathological disturbances that characterize sickle-cell anemia has been a major interest of Castle and his associates.^6,7 The chronic hemolytic anemia has been explained on the basis of the increased mechanical fragility that accompanies the sickled state,^6,8-10with diminished life span of the erythrocytes in vivo.¹¹The painful crises have been related to the increased viscosity of the blood that is proportional to the degree of sickling.^6,7Factors which impede the