The invisible string of coagulation, complement, iron, and inflammation in sickle cell disease

JD Beckman, EM Sparkenbaugh - Current opinion in hematology, 2023 - journals.lww.com
Several studies implicate dysregulation of the ADAMTS-13/VWF axis as playing a major role
in vaso-occlusive events (VOE) in SCD. Another highlight is reducing iron overload, which …

Hidden behind thromboinflammation: revealing the roles of von Willebrand factor in sickle cell disease pathophysiology

EF Vital, WA Lam - Current opinion in hematology, 2023 - journals.lww.com
The pathophysiology of SCD is multifaceted, as it involves systemwide vascular activation,
altered blood rheology, and the activation of immune responses and coagulative pathways …

VWF–ADAMTS13 axis dysfunction in children with sickle cell disease treated with hydroxycarbamide vs blood transfusion

H Fogarty, A Ahmad, F Atiq, D Doherty, S Ward… - Blood …, 2023 - ashpublications.org
Previous studies have reported elevated von Willebrand factor (VWF) levels in patients with
sickle cell disease (SCD) and demonstrated a key role for the VWF-ADAMTS13 axis in the …

Factor XII contributes to thrombotic complications and vaso-occlusion in sickle cell disease

EM Sparkenbaugh, MW Henderson, M Miller-Awe… - Blood, 2023 - ashpublications.org
A hypercoagulable state, chronic inflammation, and increased risk of venous thrombosis and
stroke are prominent features in patients with sickle cell disease (SCD). Coagulation factor …

Biased agonism of protease-activated receptor-1 regulates thromboinflammation in murine sickle cell disease

N Ramadas, K Lowder, J Dutton, F Trebak… - Blood …, 2024 - ashpublications.org
Sickle cell disease (SCD) is a hereditary hemoglobinopathy marked by hemolytic anemia
and vaso-occlusive events (VOEs). Chronic endothelial activation, inflammation, and …

Numerical study of ultra-large von Willebrand factor multimers in coagulopathy

N Rahmati, P Keshavarz Motamed… - … and Modeling in …, 2024 - Springer
An excessive von Willebrand factor (VWF) secretion, coupled with a moderate to severe
deficiency of ADAMTS13 activity, serves as a linking mechanism between inflammation to …

Mouse models of sickle cell disease: imperfect and yet very informative

S Kamimura, M Smith, S Vogel, LEF Almeida… - Blood Cells, Molecules …, 2024 - Elsevier
The root cause of sickle cell disease (SCD) has been known for nearly a century, however,
few therapies to treat the disease are available. Over several decades of work, with …

Clearance of VWF by hepatic macrophages is critical for the protective effect of ADAMTS13 in sickle cell anemia mice

H Shi, L Gao, N Kirby, B Shao, X Shan, M Kudo… - Blood, 2024 - ashpublications.org
Although it is caused by a single-nucleotide mutation in the β-globin gene, sickle cell
anemia (SCA) is a systemic disease with complex, incompletely elucidated pathologies. The …

Altered VWF: ADAMTS13 homeostasis is a target for therapeutic intervention in sickle cell disease

S Chaturvedi, MR DeBaun - Proceedings of the National …, 2022 - National Acad Sciences
Sickle cell disease (SCD) is the most common inherited blood disorder, and it affects
millions of people worldwide. In SCD, the primary event is polymerization of abnormal …

Targeting the von Willebrand Factor–ADAMTS-13 axis in sickle cell disease

P Ellsworth, EM Sparkenbaugh - Journal of Thrombosis and Haemostasis, 2023 - Elsevier
Sickle Cell Disease (SCD) is the most prevalent inherited monogenic disease worldwide,
caused by a single nucleotide mutation in the gene for beta-globin. This results in an …