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The unique red cell heterogeneity of SC disease: crystal formation, dense
reticulocytes, and unusual morphology
C Lawrence, ME Fabry and RL Nagel
Department of Medicine, Albert Einstein College of Medicine/Montefiore
Medical Center, Bronx, NY 10461.
Knowledge concerning SS (homozygous for the beta s gene) red blood cell
(RBC) heterogeneity has been useful for understanding the pathophysiology
of sickle cell anemia. No equivalent information exists for RBCs of the
compound heterozygote for the beta s and beta c genes (SC) RBCs. These RBCs
are known to be denser than most cells in normal blood and even most cells
in SS blood (Fabry et al, J Clin Invest 70:1284, 1981). We have analyzed
the characteristics of SC RBC heterogeneity and find that: (1) SC cells
exhibit unusual morphologic features, particularly the tendency for
membrane "folding" (multifolded, unifolded, and triangular shapes are all
common); (2) SC RBCs containing crystals and some containing round
hemoglobin (Hb) aggregates (billiard-ball cells) are detectable in
circulating SC blood; (3) in contrast to normal reticulocytes, which are
found mainly in a low-density RBC fraction, SC reticulocytes are found in
the densest SC RBC fraction; and (4) both deoxygenation and replacement of
extracellular Cl- by NO3- (both inhibitors of K:Cl cotransport) led to
moderate depopulation of the dense fraction and a dramatic shift of the
reticulocytes to lower density fractions. We conclude that the RBC
heterogeneity of SC disease is very different from that of SS disease. The
major contributions of properties introduced by HbC are "folded" RBCs,
intracellular crystal formation in circulating SC cells, and apparently a
very active K:Cl cotransporter that leads to unusually dense reticulocytes.
Volume 78,
Issue 8,
pp. 2104-2112,
10/15/1991
Copyright © 1991 by The American Society of Hematology
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