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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
.
Diseases
Validation
Diseases presenting
"immunodeficiency"
symptom
adrenal incidentaloma
allergic bronchopulmonary aspergillosis
cushing syndrome
dracunculiasis
hirschsprung disease
hodgkin lymphoma, classical
homocystinuria without methylmalonic aciduria
kabuki syndrome
legionellosis
malignant atrophic papulosis
oculocutaneous albinism
omenn syndrome
papillon-lefèvre syndrome
primary effusion lymphoma
primary hyperoxaluria type 1
pyomyositis
severe combined immunodeficiency
sneddon syndrome
werner syndrome
wiskott-aldrich syndrome
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
This symptom has already been validated