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Matched unrelated bone marrow transplant for Omenn syndrome.
[omenn syndrome]
Little
information
is
currently
available
on
the
outcome
and
the
long
-term
restoration
of
immune
function
in
infants
with
Omenn
syndrome
(
OS
)
treated
with
bone
marrow
transplantation
(
BMT
)
.
We
prospectively
followed
patients
with
OS
who
received
matched
unrelated
donor
(
MUD
)
BMT
at
our
center
.
Engraftment
,
immune
reconstitution
,
and
transplant-related
complications
were
recorded
.
Humoral
and
cellular
immunity
were
evaluated
.
Six
patients
with
OS
were
diagnosed
at
a
mean
age
of
4
.
6
months
and
received
a
matched
unrelated
donor
BMT
as
the
first
BMT
at
the
mean
age
of
9
.
4
months
.
All
six
patients
are
alive
and
well
at
a
mean
95
months
after
transplant
.
All
patients
have
evidence
of
full
hemopoetic
engraftment
and
robust
immune
function
.
We
have
shown
here
that
matched
unrelated
donor
BMT
is
highly
effective
in
curing
patients
with
OS
regardless
of
their
genotype
.
This
mode
of
treatment
should
be
preferred
for
patients
with
OS
when
a
related
identical
donor
is
not
available
.
Diseases
Validation
Diseases presenting
"full hemopoetic engraftment"
symptom
omenn syndrome
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