[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.
Rezaei Kalantari
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Universitè de Liège, Service d'Hématologie.
D000755:Anemia, Sickle Cell / Q000150:complications / Q000188:drug therapy*; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D000925:Anticoagulants / Q000627:therapeutic use*; D000986:Antisickling Agents / Q000627:therapeutic use*; D001120:Arginine / Q000627:therapeutic use; D001241:Aspirin / Q000627:therapeutic use; D001803:Blood Transfusion; D005440:Fluid Therapy; D018380:Hematopoietic Stem Cell Transplantation; D006461:Hemolysis; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use*; D009569:Nitric Oxide / Q000627:therapeutic use; D013923:Thromboembolism / Q000209:etiology / Q000628:therapy; D014184:Transplantation, Homologous
D000894:Anti-Inflammatory Agents, Non-Steroidal; D000925:Anticoagulants; D000986:Antisickling Agents; D009569:Nitric Oxide; D001120:Arginine; D001241:Aspirin; D006918:Hydroxyurea
Comment je traite ... une drépanocytose: actualités thérapeutiques.