The clinical effects of Hb S, an overview

Although there is as yet no definitive treatment for patients with sickle cell disease there has been considerable improvement in the overall management of these patients in recent years. This stems largely from a better understanding of the disease process. The risks from infection in infancy and childhood due to asplenia ae now well known, and children at risk ar being identified and treated. The role of platelets and the activation of clotting factors, while still controversial, are now acknowledged as appropriate areas for research. The judicious use of blood transfusions for cerebral infarction has improved the prognosis for this complication, as it has for pregnancy and the fetus. Blood transfusion, however, has its own hazards in sickle cell disease and the extent to which it can be used requires further investigation. Finally, the broad spectrum of hematological findings and individual clinical variations in sickle cell disease is now better understood.