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Thrombosis Journal
Published in: Thrombosis Journal 1/2019

Open Access 01-12-2019 | Acute Promyelocytic Leukemia | Review

Sepsis and septic shock: endothelial molecular pathogenesis associated with vascular microthrombotic disease

Author: Jae C. Chang

Published in: Thrombosis Journal | Issue 1/2019

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Abstract

In addition to protective “immune response”, sepsis is characterized by destructive “endothelial response” of the host, leading to endotheliopathy and its molecular dysfunction. Complement activation generates membrane attack complex (MAC). MAC causes channel formation to the cell membrane of pathogen, leading to death of microorganisms. In the host, MAC also may induce channel formation to innocent bystander endothelial cells (ECs) and ECs cannot be protected. This provokes endotheliopathy, which activates two independent molecular pathways: inflammatory and microthrombotic. Activated inflammatory pathway promotes the release of inflammatory cytokines and triggers inflammation. Activated microthrombotic pathway mediates platelet activation and exocytosis of unusually large von Willebrand factor multimers (ULVWF) from ECs and initiates microthrombogenesis. Excessively released ULVWF become anchored to ECs as long elongated strings and recruit activated platelets to assemble platelet-ULVWF complexes and form “microthrombi”. These microthrombi strings trigger disseminated intravascular microthrombosis (DIT), which is the underlying pathology of endotheliopathy-associated vascular microthrombotic disease (EA-VMTD). Sepsis-induced endotheliopathy promotes inflammation and DIT. Inflammation produces inflammatory response and DIT orchestrates consumptive thrombocytopenia, microangiopathic hemolytic anemia, and multiorgan dysfunction syndrome (MODS). Systemic inflammatory response syndrome (SIRS) is a combined phenotype of inflammation and endotheliopathy-associated (EA)-VMTD. Successful therapeutic design for sepsis can be achieved by counteracting the pathologic microthrombogenesis.
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Metadata
Title
Sepsis and septic shock: endothelial molecular pathogenesis associated with vascular microthrombotic disease
Author
Jae C. Chang
Publication date
01-12-2019
Publisher
BioMed Central
Published in
Thrombosis Journal / Issue 1/2019
Electronic ISSN: 1477-9560
DOI
https://doi.org/10.1186/s12959-019-0198-4

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