New insights into the pathophysiology and development of novel therapies for sickle cell disease.
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ABSTRACT:
SUBMITTER: Moerdler S
PROVIDER: S-EPMC6245971 | biostudies-literature | 2018 Nov
REPOSITORIES: biostudies-literature
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New insights into the pathophysiology and development of novel therapies for sickle cell disease.
Moerdler Scott S Manwani Deepa D
Hematology. American Society of Hematology. Education Program 20181101 1
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 t ...[more]
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