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Dense cells in sickle cell anemia: the effects of gene interaction
ME Fabry, JG Mears, P Patel, K Schaefer-Rego, LD Carmichael, G Martinez and RL Nagel
In an attempt to uncover potential genetic sources of the clinical
diversity of sickle cell anemia, we have characterized homozygous SS
patients in the following ways: percentage of dense red blood cells (% F4)
as determined from Percoll-Stractan continuous density gradients, alpha
gene deletion, average percentage of hemoglobin F (% HbF), hemoglobin in
g/dL, age, and sex. We find that alpha 4 individuals have a higher % F4
(mean 24% +/- 15%) than alpha 3 individuals (mean 12% +/- 8%) (P less than
.005). Multivariate analysis demonstrated a significant correlation among %
F4 levels and alpha-gene number and % HbF, and an interaction between the
last two variables. The other variables considered did not significantly
alter this model. As reported before, with fewer samples, we find that in
the first ten years of life of SS individuals, the frequency of alpha gene
deletion is 17%, which is comparable to that in the general black
population, while in the group over 20 years of age, the frequency rises to
49%, implying that alpha thalassemia is associated with longer survival.
These results indicate that it is necessary to consider sickle cell anemia
not only as a single gene defect, but also as a disease whose clinical
expression is the result of a group of genes capable of interacting at the
phenotypic level.
Volume 64,
Issue 5,
pp. 1042-1046,
11/01/1984
Copyright © 1984 by The American Society of Hematology
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