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Severe α-thalassemia intermedia due to a compound heterozygosity for the highly unstable Hb Adana (HBA2: c.179G>A) and a novel codon 24 (HBA2: c.75T>A) mutation.
[alpha-thalassemia]
We
report
a
novel
mutation
at
codon
24
of
the
α
2
-
globin
gene
(
HBA
2
:
c
.
75
T
 
>
 
A
)
found
in
a
Sundanese
family
.
This
novel
mutation
was
detected
during
prenatal
diagnosis
.
The
couple
already
had
a
7
-
year
-old
boy
who
exhibited
clinically
severe
α-thalassemia
intermedia
(
α-
TI
)
,
and
he
was
found
to
be
a
compound
heterozygote
for
the
novel
mutation
at
codon
24
and
the
previously
described
Hb
Adana
(
HBA
2
:
c
.
179
G
 
>
 
A
)
at
codon
59
of
the
α
2
-
globin
gene
.
The
father
was
a
carrier
of
the
novel
point
mutation
and
showed
normal
hemoglobin
(
Hb
)
and
a
low
mean
corpuscular
volume
(
MCV
)
and
mean
corpuscular
Hb
(
MCH
)
value
.
Diseases
Validation
Diseases presenting
"prenatal diagnosis"
symptom
22q11.2 deletion syndrome
achondroplasia
adrenomyeloneuropathy
alexander disease
alpha-thalassemia
aromatase deficiency
benign recurrent intrahepatic cholestasis
cadasil
canavan disease
classical phenylketonuria
cohen syndrome
congenital adrenal hyperplasia
congenital diaphragmatic hernia
congenital toxoplasmosis
cystinuria
dentinogenesis imperfecta
epidermolysis bullosa simplex
harlequin ichthyosis
holt-oram syndrome
homocystinuria without methylmalonic aciduria
hydrocephalus with stenosis of the aqueduct of sylvius
junctional epidermolysis bullosa
kindler syndrome
krabbe disease
lamellar ichthyosis
megacystis-microcolon-intestinal hypoperistalsis syndrome
monosomy 21
neonatal adrenoleukodystrophy
oculocutaneous albinism
omenn syndrome
phenylketonuria
primary hyperoxaluria type 1
pyruvate dehydrogenase deficiency
severe combined immunodeficiency
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
zellweger syndrome
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