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Novel adopted immunotherapy for mixed chimerism after unrelated cord blood transplantation in Omenn syndrome.
[omenn syndrome]
Omenn
syndrome
is
a
variant
form
of
severe
combined
immunodeficiency
.
It
is
fatal
unless
treated
by
allogeneic
stem
cell
transplantation
(
SCT
)
,
which
is
the
only
curative
approach
.
However
,
both
treatment-related
complications
and
graft
rejection
are
major
obstacles
to
treatment
success
.
This
report
describes
a
case
with
Omenn
syndrome
who
developed
mixed
chimerism
after
unrelated
cord
blood
transplantation
(
UCBT
)
.
This
case
was
successfully
treated
by
altering
the
patient
's
immunosuppression
and
donor
lymphocyte
infusion
(
DLI
)
with
donor
cord
blood
-derived
activated
CD
4
+
T
cells
ex
vivo
expanded
from
the
cord
blood
cell
residues
in
an
infused
bag
.
This
novel
development
to
expand
CD
4
+
T
-
lymphocytes
from
the
donor
cord
blood
unit
for
the
use
of
DLI
would
serve
as
a
useful
method
to
overcome
the
risk
of
graft
rejection
in
SCT
for
primary
immunodeficiency
disorders
with
residual
cell-mediated
immunity
without
compounding
graft-
vs
.
-
host
disease
,
especially
in
the
UCBT
setting
.