THE PATHOLOGY OF SICKLE CELL DISEASE

Abstract

The hemolytic crises which characterize sickle cell disease are caused by multiple infarctions, visceral and skeletal. Thrombi and disintegration of the sickled cells occur giving rise to the hemolytic aspects of the disease. The pathologic processes have their origin in the infarcts. Each susceptible cell has a threshold for sickling which is detd. by its O2 concn. As the cell ages the threshold lowers until the cell becomes permanently sickled in the circulation. A sickled cell passes through capillaries with difficulty because of its altered physical characteristics. These abnormally shaped cells accumulate and eventually cause mechanical obstruction and thrombosis of capillaries. Coagulating bodies are thus liberated into the circulation which accelerate the process eventuating in a crisis. After a crisis the circulation is "rejuvenated" by liberation of young cells.