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

Scott Moerdler, Deepa G. Manwani

Research output: Contribution to journalReview article

3 Citations (Scopus)

Abstract

Although the seminal event in sickle cell disease is the polymerization of abnormal hemoglobin, the downstream pathophysiology of vasoocclusion results from heterotypic 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. American Society of Hematology. Education Program
Volume2018
Issue number1
DOIs
StatePublished - Nov 30 2018

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Abnormal Hemoglobins
Sickle Cell Anemia
Hemostatics
Hemolysis
Reperfusion Injury
Oxidants
Polymerization
Adhesives
Endothelium
Neutrophils
Blood Platelets
Erythrocytes
Inflammation
Therapeutics

ASJC Scopus subject areas

  • Hematology

Cite this

New insights into the pathophysiology and development of novel therapies for sickle cell disease. / Moerdler, Scott; Manwani, Deepa G.

In: Hematology. American Society of Hematology. Education Program, Vol. 2018, No. 1, 30.11.2018, p. 493-506.

Research output: Contribution to journalReview article

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