Fig. 5.
Maximum RBC deformability as a function of cell age in unsplenectomized and splenectomized HS patients with band 3 deficiency and spectrin/ankyrin deficiency.
(A) Band 3 deficiency. RBCs from unsplenectomized patients AZ1, AZ3, AZ8, WR4 (open rectangles). RBCs from splenectomized family members AZ2, AZ4, AZ9, WR5 (closed rectangles). In 3 of these patients, only 2 data points are included because the amount of RBCs recovered from denser fractions allowed band 4.1a/4.1b ratio determination but not ektacytometry. Band 4.1a/4.1b ratios did not significantly increase in denser fractions (Figure 4), suggesting a similarly rapid decay of maximum deformability in denser fractions as found for the lighter 2 fractions. (B) Spectrin/ankyrin deficiency. RBCs from unsplenectomized patients AZ15, AZ17, HL1 (open triangles). RBCs from splenectomized family members AZ16, AZ18, HL2 (closed triangles) and RBCs from 2 unpaired, splenectomized patients (HE and EG 6.2, filled diamonds). Results from control RBCs are shown in both graphs with open circles.