Standardized microfluidic assessment of red blood cell-mediated microcapillary occlusion: Association with clinical phenotype and hydroxyurea responsiveness in sickle cell disease.

ABSTRACT:

Objectives

We present a standardized in vitro microfluidic assay and Occlusion Index (OI) for the assessment of red blood cell (RBC)-mediated microcapillary occlusion and its clinical associations in sickle cell disease (SCD).

Methods

Red blood cell mediated microcapillary occlusion represented by OI and its clinical associations were assessed for seven subjects with hemoglobin-SC disease (HbSC), 18 subjects with homozygous SCD (HbSS), and five control individuals (HbAA).

Results

We identified two sub-populations with HbSS based on the OI distribution. HbSS subjects with relatively higher OIs had significantly lower hemoglobin levels, lower fetal hemoglobin (HbF) levels, and lower mean corpuscular volume (MCV), but significantly higher serum lactate dehydrogenase leve

SUBMITTER: Man Y 

PROVIDER: S-EPMC7920910 | biostudies-literature | 2021 Feb

REPOSITORIES: biostudies-literature