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Hematopoietic stem cell transplantation in Omenn syndrome: a single-center experience.
[omenn syndrome]
We
retrospectively
analyzed
the
outcome
of
hematopoietic
stem
cell
transplantations
(
HSCT
)
performed
at
our
Center
between
1991
and
2002
in
11
unselected
patients
with
Omenn
syndrome
,
a
variant
of
severe
combined
immunodeficiency
.
The
patients
'
mean
age
at
the
time
of
the
first
HSCT
was
8
.
4
months
.
Two
patients
received
two
,
and
one
patient
three
,
HSCT
procedures
.
The
resulting
15
HSCT
derived
in
seven
cases
from
HLA-haploidentical
parents
,
in
four
patients
from
matched
unrelated
donors
,
in
three
cases
from
an
HLA
phenotypically
identical
related
donor
,
and
in
one
case
from
an
HLA
genotypically
identical
family
donor
.
Nine
out
of
11
patients
are
alive
and
immunoreconstituted
30
-
146
months
after
transplantation
.
At
the
time
of
the
most
recent
evaluation
,
all
of
the
nine
survivors
had
normal
T
-
cell
function
,
and
eight
of
them
had
developed
normal
antibody
production
.
This
study
demonstrates
an
overall
mortality
of
18
.
2
%
,
which
is
substantially
lower
than
previously
reported
.
Early
recognition
of
OS
,
rapid
initiation
of
adequate
supportive
treatment
and
HSCT
lead
to
improved
outcome
for
this
otherwise
fatal
disease
,
regardless
of
the
origin
and
matching
of
hematopoietic
stem
cells
.
Diseases
Validation
Diseases presenting
"developed normal antibody production. this study"
symptom
omenn syndrome
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