Fetal characteristics of erythrocytes in sickle cell anemia: an
immunofluorescence study of individual cells
A Maniatis, T Papayannopoulou and JF Bertles
In a group of disease states that includes sickle cell anemia (SS disease),
two fetal erythrocyte markers, Hb F and i antigen, persist into adulthood.
Using the technique of single-cell immunofluorescence, we determined the
expression of l-i antigens and the presence of Hb F within populations of
erythrocytes. Subjects tested included normal adults, normal newborns,
patients with SS disease, and individuals with sickle cell trait. We
classified erythrocytes reacting to anti-i as i cells and those reacting to
anti-l as l cells, a terminology analogous to that used to identify
erythrocytes containing increased amounts of Hb F as F cells. The
expression of l and i antigens within populations of both normal and SS
erythrocytes was found to be heterogeneous. The proporations of both i
cells and l cells in all SS patients studied exceeded those found in normal
adults, and an overall stronger-than- normal reactivity of individual SS
cells to the two antibodies was observed. Proportions of F cells showed no
correlation with proportions of i cells; and with double fluroescence
staining for both Hb F and i, a significant proportion of each total SS red
cell population was found to carry only one or the other marker. These
findings confirm and clarify on a cellular level our previous
demonstration, by means of quantitative hemagglutination, that there is
increased expression of both l and i by whole populations of SS
erythrocytes. In addition, we provide here new information on the
expression of l and i within populations of normal human erythrocytes.
Volume 54,
Issue 1,
pp. 159-168,
07/01/1979
Copyright © 1979 by The American Society of Hematology