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Insights into determinants of spleen injury in sickle cell anemia

Sara El Hoss, Sylvie Cochet, Mickaël Marin, Claudine Lapouméroulie, Michael Dussiot, Naïm Bouazza, Caroline Elie, Mariane de Montalembert, Cécile Arnaud, Corinne Guitton, Béatrice Pellegrino, Marie Hélène Odièvre, Frédérique Moati, Caroline Le Van Kim, Yves Colin Aronovicz, Wassim El Nemer and Valentine Brousse

Data supplements

Article Figures & Data

Figures

  • Figure 1.

    HJB-RBCs and ISCs in children with SCA. (A) %HJB-RBCs determined by IFC in 45 children at 3 to 6 and 18 months. ****P < .0001, Wilcoxon paired test. (B) Distribution of %HJB-RBCs with respect to splenic uptake as measured by 99mTc heated RBCs spleen scintigraphy. Splenic uptake of 100% to 150% (n = 15), 75% to 100% (n = 4), 50% to 75% (n = 1), 25% to 50% (n = 2), and 0% to 25% (n = 5). (C) %ISCs determined by IFC in 45 children at 3 to 6 and 18 months. ****P < .0001, Wilcoxon paired test. (D) Spearman correlation between %ISCs and %HJB-RBCs at 3 to 6 (red dots) and 18 months (blue dots). n = 99; R2 = 0.69; ***P < .001.

  • Figure 2.

    Lu/BCAM expression and mediated RBC adhesion. (A) Microscopic images of RBCs adhering to TrHBMEC monolayers and laminin 521-coated microchannels at 3 to 6 and 24 months. Original magnification ×20. (B-C) The amount of adherent RBCs/mm2 on TrHBMEC-coated channels (n = 15) (B) and laminin-coated channels (n = 15) (C) at 3 to 6, 12, 18, and 24 months. *P < .05, **P < .005, ***P < .001, Wilcoxon test. (D) Mean fluorescence intensity of Lu/BCAM on mature RBCs. ****P < .0001, Wilcoxon test.

  • Figure 3.

    RBC adhesion and HJB-RBCs in children with SCA. Spearman correlation between the amount of adherent RBCs/mm2 on laminin (A) and TrHBMEC and %HJB-RBCs (B). At 3 to 6 (red dots) and 18 months (blue dots). n = 28; R2 = 0.43; ***P < .001.

  • Figure 4.

    HJB-RBCs and ISCs in asymptomatic and ASS-affected infants. Comparison of %HJB-RBCs (A) and %ISCs (B) at 3 to 6 and 18 months in asymptomatic (n = 22) and ASS infants (n = 7, for whom samples were available for analysis). **P < .005, Wilcoxon test.

Tables

  • Table 1.

    Spleen function at 3-6 and 18 months of age in children with SCA, as measured by 99mTc-heated RBC spleen scintigraphy (splenic uptake) and %HJB-RBCs

    Age, moSplenic uptake, %Patients, n (%)%HJB-RBCs, median (range)
    3-6100-15017 (68)0.32 (0.01-1.27)
    75-1003 (12)0.16, 0.3 and 1.96
    50-751 (4)ND
    25-502 (8)0.05 and 3.18
    0-252 (8)2.88 and 2.9
    18100-1509 (50)0.07 (0.01-1.3)
    75-1002 (11.1)0.8 and 1.41
    50-751 (5.6)1.12
    25-502 (11.1)2.98*
    0-254 (22.2)4.77 (1.37-5.11)
    • ND, not determined.

    • * Only 1 blood sample was processed.