Red cell abnormalities characterized by ektacytometry in children with cholestasis

BackgroundSpur-cell anemia sometimes accompanies cholestasis. We postulated that even in the absence of spur-cells, cholestasis might alter red blood cell (RBC) osmotic fragility and deformability. Therefore, we assessed these RBC measures by ektacytometry in pediatric patients.MethodsWe conducted a single center, prospective, cross-sectional investigation of RBC membrane characteristics by ektacytometry in pediatric patients with intra- and extrahepatic cholestasis followed at Cincinnati Children's Hospital Medical Center. We measured red cell membrane fragility and deformability in 17 patients with cholestasis and 17 age-matched controls without cholestasis.ResultsPatients with cholestasis had decreased RBC osmotic fragility compared to controls, with a significant left shift in Omin, indicating increased RBC surface-to-volume ratio. One showed spur cell morphology. However, the other 16 had no spurring, indicating that ektacytometry is a sensitive method to detect RBC membrane abnormalities. Left shift of Omin positively correlated with serum conjugated bilirubin levels and even more negatively with serum vitamin E concentration.ConclusionsThis study suggests that subclinical red blood cell membrane abnormalities exist in most pediatric patients with cholestasis, increasing risk for hemolysis when subjected to oxidative stress. Hence minimizing pro-oxidants exposure and maximizing antioxidant exposure is advisable for this group.ClinicalTrials.gov IdentifierNCT05582447 https://clinicaltrials.gov/ct2/show/NCT05582447?cond=Cholestasis&cntry=US&state=US%3AOH&city=Cincinnati&draw=2&rank=2.ImpactSpur cell anemia due to decreased red cell osmotic fragility and decreased deformability has been reported among patients with cholestasis.Ektacytometry is a reliable, reproducible method to measure red cell osmotic fragility and deformability.Few data describe red cell osmotic fragility or deformability in patients with cholestasis who may or may not have spur cell anemia.Ektacytometry shows that red cell osmotic fragility and deformability are decreased in many children with cholestasis even when spur cell anemia has not yet occurred.Factors associated with decreased osmotic fragility include elevated serum bilirubin, elevated serum bile acids, and decreased serum vitamin E.

Automated summary

This study investigates the impact of cholestasis on red blood cell osmotic fragility and deformability in pediatric patients. It shows that even in the absence of spur cells, cholestasis can alter these red blood cell measures, increasing the risk of hemolysis under oxidative stress. The findings suggest the importance of minimizing pro-oxidant exposure and maximizing antioxidant exposure in this patient population.