Elevated vWF with Normal ADAMTS13 in Pediatric Sickle Cell Disease: Links to Crises and Ischemic Stroke Open Access

1. Elevated vWF with normal ADAMTS13 levels observed in VOC and ischemic stroke in pediatric SCD, accompanied with inflammation and hemolysis.

2. Hematologic and endothelial markers may serve as alternatives to imaging for detecting high-risk SCD complications in low-resource settings

Sickle cell disease (SCD) remains a significant healthcare challenge with sub-Saharan Africa being the most affected. The disease is characterized by complications including Vaso occlusive crisis and ischemic stroke leading to high morbidity and mortality rates. In under-resourced communities, inaccessibility of MRI and CT scanning complicates screening hence a need for hematological assays as accessible cost-effective alternatives to bridge the gap. The current cross-sectional study involving 80 pediatric SCD patients and 32 normal controls focused on vWF-ADAMTS13 axis, believed to be involved in vaso-occlusion and ischemia in SCD. Through hematological assays, the study evaluated FBC, vWF antigen and activity, and ADAMTS13 antigen levels in SCD patients and healthy controls. The study revealed elevated neutrophil counts coupled with reduced RBC counts, lower hemoglobin levels, elevated nucleated RBCs, and lower platelet counts in pediatric SCD phenotypes compared to healthy controls. VOC cases were associated with elevated vWF antigen, normal vWF activity, and normal ADAMTS13 antigen while ischemic stroke cases showed no significant differences in VWF antigen and activity, and ADAMTS13 antigen levels compared to the normal controls. Comparatively, SCD in steady state had elevated ADAMTS13 antigen levels compared to healthy controls. These findings indicate chronic inflammation and hemolysis, likely sustained by neutrophil-linked proinflammatory mediators, might be involved in dysregulation of vWF-ADAMTS13 axis, increasing the risk of vaso-occlusion. Therefore, FBC, VWF:Ag, VWF:Act, and ADAMTS13 antigen may be used for risk stratification and early diagnosis of pediatric the SCD complications in resource-limited settings.