[Emergencies in adults with sickle cell disease]

Sickle cell disease is an inherited disease characterized by the presence of an abnormal haemoglobin. It is the more prevalent genetic disease at birth in Ile-de France area. Sickle cell disease can be complicated by acute vaso-occlusive crisis, chronic visceral involvement related to ischemic process, and infectious complications. In adults, acute vaso-occlusive crisis is the major clinical problem prompting admission to hospital and the main cause of death. It mainly manifests by osteoarticular pain and acute chest syndrome and can be complicated by multiorgan failure. Acute vaso-occlusive crisis can be also manifested by priapism, ischemic or haemorrhagic stroke, or abdominal pain. The main treatment of severe acute vaso-occlusive crisis is based on transfusion.