The systems biology-based argument for taking a bold step in chemoprophylaxis of sickle vasculopathy (pages 543–545)

摘要

The chronic vasculopathy of sickle cell disease [1,2] is anparticularly deadly complication of sickle pathobiology. Itnresults in—or at least contributes to—stroke syndromes,npulmonary hypertension, priapism, fetal wastage andngrowth retardation, autosplenectomy, and chronic renal disease.nAlthough these disease complications have beennknown for years, only recently it was suggested that thesenare all linked by a common pathogenesis, and that thenpathologic lesion of sickle vasculopathy is similar to that ofnatherosclerosis in the general population (except for thenabsence of a fatty streak in sickle disease) [1]. The reportnof the NIH Workshop on Sickle Vasculopathy that is presentednin this issue of the American Journal of Hematologynbriefly describes the overall pathophysiology of this particularnaspect of sickle cell disease, as currently understood byninvestigators of these problems. For this information, I refernreaders to the workshop summary [3]. And a much morendetailed consideration of this problem is presented elsewheren[2]. However, I do wish to emphasize three criticalnpoints.