New insights into the pathophysiology and development of novel therapies for sickle cell disease

Scott Moerdler, Deepa Manwani

Research output: Contribution to journalReview article

5 Scopus citations

Abstract

Although the seminal event in sickle cell disease is the polymerization of abnormal hemoglobin, the downstream pathophysiology of vasoocclusion results fromheterotypic interactions between the altered, adhesive sickle cell red blood cells, neutrophils, endothelium, and platelets. Ischemia reperfusion injury, hemolysis, and oxidant damage all contribute to heightened inflammation and activation of the hemostatic system. These various pathways are the focus of emerging treatments with potential to ameliorate disease manifestations. This review summarizes the considerable progress in development of these agents despite challenges in selection of study end points and complex pathophysiology.

Original languageEnglish (US)
Pages (from-to)493-506
Number of pages14
JournalHematology / the Education Program of the American Society of Hematology. American Society of Hematology. Education Program
Volume2018
Issue number1
DOIs
StatePublished - Nov 30 2018

ASJC Scopus subject areas

  • Hematology

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