ABSTRACT
Sickle cell anaemia is a hereditary autosomal recessive disorder characterized by chronic haemolytic anaemia, painful episodes due to the occlusion of small vessels and an increased susceptibility to severe, often fatal infections. Vaso-occlusive episodes are related to the tendency of deoxygenated HbS to undergo polymerization, a phenomenon inducing red cell sickling, membrane alterations, haemolysis and changes in the rheology of the red cells. As a consequence, episodic attacks of ischaemic pain and infarcts occur in various organs: bones and joints, the abdomen, the chest, the central nervous system, the kidneys and the eyes, to mention only the organs most frequently involved. Table 19.1 summarizes the primary causes of death from sickle cell disease.
