Hidden behind thromboinflammation: revealing the roles of von Willebrand factor in sickle cell disease pathophysiology
Year: 2023
Type: review
Abstract: PURPOSE OF REVIEW: This review provides an update on the pathophysiology of sickle cell disease (SCD) with a particular focus on the dysregulation of the von Willebrand factor (VWF) - ADAMTS13 axis that contributes to its pathogenesis. In discussing recent developments, we hope to encourage new and ongoing discussions surrounding therapeutic targets for SCD. RECENT FINDINGS: Within the last 5 years, the role of VWF in the pathophysiology of SCD has been further elucidated and is now a target of study in ongoing clinical trials. SUMMARY: The pathophysiology of SCD is multifaceted, as it involves systemwide vascular activation, altered blood rheology, and the activation of immune responses and coagulative pathways. The presence of VWF in excess in SCD, particularly in its largest multimeric form, greatly contributes to its pathogenesis. Understanding the molecular mechanisms that underly the presence of large VWF multimers in SCD will provide further insight into the pathogenesis of SCD and provide specific targets for therapy.
Source: Current Opinion in Hematology
Authors: Eudorah F. Vital, Wilbur A. Lam
Language: English
Subfield: Genetics (clinical)
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
Awards: A New Paradigm to Treat Bleeding by Augmenting Hemostasis via Microscale Electrical Fields, Redefining Clinical Viscosity in Sickle Cell Diseaseby Leveraging Microfluidic Technologies, Engineering biophysical microtechnologies for hematologic applications in health and disease, Medical Scientist Training Program
OpenAlex ID: W4322616900