[Management of sickle cell disease]

Hypoxia, hemolysis and infection are more or less associated in patients affected with sickle cell disease. Treatment is based on a programme including regular lifestyle, hydration, folic acid supply, prevention of pneumococcal infections and cerebrovascular events in children, regular follow-up in specialised centres allowing precocious screening and treatment of organ deficiency. Some patients exhibit a severe form and need intensive preventive care, such as chronic transfusion, hydroxyurea or bone marrow transplantation for children and adolescent with an HLA-identical sibling. The choice between these strategies is multifactorial, excepted in patients with a severe cerebral vasculopathy, for whom chronic transfusion or bone marrow transplantation are preferable. We usually propose hydroxyurea as a first line treatment to patients with recurrent pain crises or acute chest syndromes. In cases of refusal, initial or secondary failure (which occur more frequently in adults), or intolerance of hydroxyurea, patients with a severe disease are chronically transfused, which leads them most of the time to necessitate iron chelation.