Sickle Cell Disease Biofluid Chip Technology (SCD BioChip)

Study Summary

'Sickle-shaped' anemia was first clinically described in the US in 1910, and the mutated heritable sickle hemoglobin molecule was identified in 1949. The pathophysiology of SCD is a consequence of abnormal polymerization of sickle hemoglobin (HbS) and its effects on red cell membrane properties, shape, and density, and subsequent critical changes in inflammatory cell and endothelial cell function. Our goal is to understand the impact of CMA abnormalities in SCD, by interrogating a number of recognized interactions in a range of clinical phenotypes. To date, correlative studies in SCD, by us and others, have range between clinical reports, based on tests, interventions, and chart review of individuals or groups of individuals and, at the other extreme, identification of functional gene polymorphisms based on population studies. The investigators wish to augment these studies through a systematic examination of cellular membrane properties and activation status. Of hematologic disorders, SCD may be unusually susceptible to such an examination.

Primary Outcome

Red and white cell adhesion to biomolecules (e.g. laminin, fibronectin, and selectins) will be measured on a microfluidic device, the SCD Biochip. Adhesion will be measured under normal oxygen and low oxygen conditions. Adhesions will be assessed relative to clinical findings, such as hematology parameters, and evidence for vaso-occlusion, pain, inflammation, and vasculopathy in patients with sickle cell disease.endothelial and hematopoietic precursor cells based on membrane properties and adhes

Conditions Studied

Interventions

• OTHER SCD Group

Study Locations (1)