The treatment of sickle cell disease. A historical and chronological literature review of the therapies applied since 1910.
The available literature is reviewed with reference to earlier and present therapies. When possible, methods used in clinical trials with different substances are briefly described, also giving their historical background. The substances studied or methods used are reported in chronological order. A total of 117 references have been made. Of these only 14 reported controlled double blind clinical trials (5 with urea, 3 with alkalines, 2 with phenothiazines, 2 with antisludging agents and one each with the defibrinating agent Arvin and cyanate). The controlled trials could not confirm the benefits claimed for the substances in the prevention or treatment of sickle cell crises. Treatment of the crises is still symptomatic and conventional.