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X-linked dominant control of F-cells in normal adult life: characterization
of the Swiss type as hereditary persistence of fetal hemoglobin regulated
dominantly by gene(s) on X chromosome
K Miyoshi, Y Kaneto, H Kawai, H Ohchi, S Niki, K Hasegawa, A Shirakami and T Yamano
First Department of Internal Medicine, School of Medicine, University of
Tokushima, Japan.
Fetal hemoglobin (HbF) levels determined in healthy Japanese adults ranged
from 0.3% to 16.0% as F cells and 0.17% to 2.28% as HbF content, which were
the same as those obtained in other countries. The frequency distribution
of 300 healthy adults with various numbers of F cells consisted
statistically of two different groups, low and high F-cell groups.
Individuals with greater than or equal to 4.4% of F cells (HbF about 0.7%)
were defined as the high F-cell trait, which accounted for 11.3% of males
and 20.7% of females. Family studies of 21 probands with this trait and
sex-different frequency analyses in the population and probands revealed
X-linked dominant inheritance. Two other families of the trait associated
with color blindness were described, although no definitive evidence for
linkage was obtained between the two. A review of population and family
studies reported in the literature indicated that persons with Swiss-type
hereditary persistence of fetal hemoglobin (HPFH) are of the same kind as
this trait in their incidence and inheritance form, but represent a portion
of the trait with higher levels of HbF or F cells. The existence of X
chromosome-localized regulatory gene(s) for the developmental switch of
human Hb production is discussed.
Volume 72,
Issue 6,
pp. 1854-1860,
12/01/1988
Copyright © 1988 by The American Society of Hematology
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