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Sickle cell anemia and α-thalassemia: A modulating factor in homozygous HbS/S patients in Oman.
[alpha-thalassemia]
We
report
the
general
phenotype
severity
and
the
hematological
presentation
in
a
cohort
of
125
sickle
cell
anemia
(
SCA
)
patients
with
identical
homozygous
HbS
/
S
genotype
and
categorized
by
identical
β
(
S
)
haplotype
,
both
with
and
without
alpha
thalassemia
.
No
clear
general
phenotype
correlation
was
found
when
patients
were
compared
regardless
of
the
haplotype
but
overall
,
patients
with
homozygous
alpha
thalassemia
(
α-
/
α-
)
had
the
highest
Hb
,
HCT
,
RBC
and
the
lowest
MCV
,
MCH
and
MCHC
levels
.
When
patients
with
identical
haplotype
were
compared
,
the
mildest
hematological
and
clinical
conditions
were
observed
in
patients
of
the
Asian
/
Asian
haplotype
,
also
known
as
Arab-
Indian
haplotype
,
and
carriers
of
α-thalassemia
,
suggesting
an
additional
ameliorating
effect
of
alpha
thalassemia
.
In
conclusion
,
our
results
show
that
alpha
thalassemia
improves
the
hematological
conditions
but
amelioration
of
the
general
disease
severity
is
only
noticed
when
compared
in
cohorts
of
the
same
haplotype
.
Diseases
Validation
Diseases presenting
"clear general phenotype correlation"
symptom
alpha-thalassemia
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