Hidden behind thromboinflammation: revealing the roles of von Willebrand factor in sickle cell disease pathophysiology.
Curr Opin Hematol
; 30(3): 86-92, 2023 05 01.
Article
em En
| MEDLINE
| ID: mdl-36853830
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.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Trombose
/
Anemia Falciforme
Tipo de estudo:
Etiology_studies
Limite:
Humans
Idioma:
En
Revista:
Curr Opin Hematol
Assunto da revista:
HEMATOLOGIA
Ano de publicação:
2023
Tipo de documento:
Article