The life span of the sickle cell and the pathogenesis of sickle cell anemia.

Abstract

Abstract The method of cross determination of the survival time of red cells was used for the study of the pathogenesis of sickle cell anemia. When normal erythrocytes transfused into a recipient with a hemolytic syndrome survive normally, whereas the recipient's own red cells transfused into a normal person, have a considerably shortened life span, an intracorpuscular abnormality may be suspected. Contrariwise, when normal red cells transfused into the patient with the hemolytic syndrome are as rapidly destroyed as the patient's own cells, the presence of an indiscriminating extracorpuscular mechanism may be assumed. In this study, trait cells were transfused into patients with sickle cell anemia and anemia cells into healthy recipients harboring the sickle cell trait. Trait cells survive normally (120 days) when transfused into patients with sickle cell anemia, whereas the patients' own cells continue to be hemolyzed at a faster rate. Anemia cells, when transfused into trait carriers, have a shortened life span with an average of about one-fourth of the normal. Therefore the pathogenic principle operating in sickle cell anemia resides within the red cells themselves; no extracorpuscular mechanism can apparently be made responsible for this hemolytic syndrome. Trait cells can be used safely for therapeutic transfusions. An analysis of the known facts about the sickling phenomenon shows that the sickling process which is the expression of an abnormality of the stroma does not lend itself to a satisfactory explanation of the pathogenesis of the anemia. The hypothesis is formulated that sickle cell anemia develops because of an additional alteration in the cyto-skeleton which is qualitatively different from the structural anomaly responsible for the sickling phenomenon.