Cohort study reveals fewer painful crises and lower mortality in sickle cell patients with reduced red cell deformability, suggesting cellular rheology modulates disease severity.
Key Points
To evaluate whether red blood cell deformability and dense cell count modulate the frequency of painful crises and clinical severity in homozygous sickle cell anemia.
Stratified adult homozygous SS patients without alpha-gene deletion and with steady-state Hb F < 6.0% into low (≤37% of control) versus high (>65% of control) RBC deformability groups.
Followed each patient for a minimum of 3 years, measuring RBC deformability index (DI 290) via ektacytometry and quantifying dense cells on a discontinuous Stractan gradient.
Patients with low RBC deformability had significantly fewer painful crises and a higher proportion of dense cells (mean 22.2% vs. 9.8% of total circulating cells) than those with high deformability.
The low deformability group experienced zero deaths and a higher incidence of leg ulcers, whereas the high deformability group had a 33% mortality rate during follow-up.