MIRCA readouts capture low red cell deformability in individuals on chronic transfusion therapy, which can be improved with the in vitro addition of osivelotor or voxelotor. (A-D) A nonlinear relationship of HbS% with MIRCA (A-B) and LoRRca (C-D) readouts. NOI and HOI values worsen, EI max values remain stable, and EI Min values improve as HbS values decrease below 40% after blood transfusion. The statistically significant association of HOI and HbS (P = .001) could be due to lower HbF values in this group compared with the patients with HbS between 40 and 80 (median HbF = 0% [range, 0-9.3] vs 12.2% [range, 6.5-20], P = .013). (E-F) No significant difference in the median NOI (E) and HOI (F) of individuals with HbS <40% vs those with HbS >90% and not on HU MTD (P = .3 and P = .3, respectively). To test the impact of in vitro addition of antisickling agents on deformability, RBCs from 12 individuals with HbSS were treated with 1.66 mM osivelotor (n = 8) or voxelotor (n = 6) (G) dissolved in DMSO or with DMSO alone, incubated at 25°C for 1 hour and resuspended in 1.5% MBS in 1× PBS and run on the MIRCA. Osivelotor- and voxelotor-treated samples were found to have 17.3% and 10.8% lower HOI values compared with DMSO-treated controls (P = .008 and .03, respectively, Wilcoxon signed rank test). However, osivelotor and voxelotor sample HOI values were still elevated by 3.7% and 7.0% compared with 4 healthy controls (P = .008 and .006, respectively, Mann-Whitney U test). ∗P ≤ .05; ∗∗P ≤ .01. ns, not significant.
Figure 3.

MIRCA readouts capture low red cell deformability in individuals on chronic transfusion therapy, which can be improved with the in vitro addition of osivelotor or voxelotor. (A-D) A nonlinear relationship of HbS% with MIRCA (A-B) and LoRRca (C-D) readouts. NOI and HOI values worsen, EI max values remain stable, and EI Min values improve as HbS values decrease below 40% after blood transfusion. The statistically significant association of HOI and HbS (P = .001) could be due to lower HbF values in this group compared with the patients with HbS between 40 and 80 (median HbF = 0% [range, 0-9.3] vs 12.2% [range, 6.5-20], P = .013). (E-F) No significant difference in the median NOI (E) and HOI (F) of individuals with HbS <40% vs those with HbS >90% and not on HU MTD (P = .3 and P = .3, respectively). To test the impact of in vitro addition of antisickling agents on deformability, RBCs from 12 individuals with HbSS were treated with 1.66 mM osivelotor (n = 8) or voxelotor (n = 6) (G) dissolved in DMSO or with DMSO alone, incubated at 25°C for 1 hour and resuspended in 1.5% MBS in 1× PBS and run on the MIRCA. Osivelotor- and voxelotor-treated samples were found to have 17.3% and 10.8% lower HOI values compared with DMSO-treated controls (P = .008 and .03, respectively, Wilcoxon signed rank test). However, osivelotor and voxelotor sample HOI values were still elevated by 3.7% and 7.0% compared with 4 healthy controls (P = .008 and .006, respectively, Mann-Whitney U test). ∗P ≤ .05; ∗∗P ≤ .01. ns, not significant.

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