[Treatment of major sickle cell syndromes. The point of view of the internist]

Abstract

Major sickle cell syndromes constitute at present the most important risk of genetic disease in the wider Paris area. Sickle cell patients are exposed to vaso-occlusive attacks and to chronic visceral complications of ischemic origin capable of affecting all organs. Acute vaso-occlusive complications represent the first cause of hospitalisation and the main cause of death for the adult patient. Functional asplenia also occurs with a risk of infection through encapsuled germs. The risk of infection must always be taken into account by clinicians treating adults even if serious infectious accidents are less frequent in adults than in children. Sickle cell disease is a public health problem. Better knowledge of acute vaso-occlusive complications and ways of treating them rapidly by simple symptomatic measures mean that the mortality rate and the risk of serious functional sequelae can be decreased. Such measures allow sickle cell patients to enjoy a life expectancy beyond 50 years.