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 …

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 …

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 …