[How I treat ... sickle cell anemia: current therapies]

Vaso-occlusive crisis, thromboembolic events and acute chest syndrome are the most frequent complications in patients with sickle cell disease. Appropriate management of the vaso-occlusive painful crisis includes aggressive analgesia and hydratation. For thromboembolic events, anticoagulation and acetylsalicylic acid with dipyridamole may be considered. Transfusion has a place during some crisis, when hemolysis is increased. Hydroxyurea, a drug that induce foetal hemoglobin synthesis until 30%, has a therapeutic benefit in decreasing the number of vaso-occlusive episodes, of transfusions and that of the acute chest syndrome. Currently, the only treatment that allows definitive cures is familial allogeneic stem cell transplantation. New agents are currently under clinical evaluation such as NO and arginine for aggressive crisis or other aggressive complications and administration magnesium and clotrimazole to prevent intracellular deshydratation. These drugs block cation-transport channels in erythrocyte membranes.