Is sickle cell disease-related neurotoxicity a systemic endotheliopathy?

The aim of the present article is to review the role of endothelial damage and dysfunction in the vaso-occlusive episodes associated with sickle cell disease (SCD). This inherited hematological disorder leads to irreversible damage of multiple organs through a wide variety of mechanisms, such as sickling of red cells, oxidative state due to ischemic-reperfusion episodes, inflammation, hypercoagulation state, and platelet activation, among others. In SCD, the endothelium arises as the key entity where most of these processes, which eventually lead to increased morbidly and mortality, interact. This review begins with the already accepted idea that organ-specific vasculopathy precedes clinical manifestation, and briefly explains one of the main triggers of vaso-occlusive episodes, the complex interplay between blood cells and the dysfunctional endothelium. Endothelial protective strategies emerge as a potential tool for the prevention of organ-specific disease in SCD. Actually, this knowledge is currently used for the development of potential pharmacologic interventions to improve the lives of SCD patients.

Informations de copyright

Copyright © 2020 King Faisal Specialist Hospital & Research Centre. Published by Elsevier Ltd. All rights reserved.

Déclaration de conflit d'intérêts

Declaration of Competing Interest MP and MDR declare a conflict of interest with Jazz Pharmaceuticals Plc/Gentium Inc in the form of speaker’s fee for symposiums, and EC declares a conflict of interest with Jazz Pharmaceuticals plc/Gentium Inc as consultant and in the form of speaker’s fee for symposiums.