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Bone marrow transplantation without conditioning regimen in Omenn syndrome: a case report.
[omenn syndrome]
OS
is
a
non-
SCID
immunodeficiency
characterized
by
a
poor
outcome
even
after
BMT
.
We
report
here
a
case
of
BMT
without
preparative
conditioning
regimen
,
and
with
a
successful
engraftment
in
a
five
-
month
-old
infant
with
OS
.
The
patient
was
transplanted
with
15
x
10
(
8
)
bone
marrow
mononuclear
cells
/
kg
,
from
his
HLA
matched
brother
,
without
preparative
regimen
and
GVHD
prophylaxis
.
Immunological
status
was
assessed
before
and
after
the
BMT
,
and
the
engraftment
was
monitored
with
microchimerism
analysis
.
Six
days
after
BMT
,
an
acute
GVHD
involving
first
the
skin
,
then
the
liver
and
gut
,
complicated
the
post-transplantation
course
.
An
excellent
engraftment
was
confirmed
by
donor
chimerism
over
95
%
respectively
at
day
post-transplantation
30
,
60
,
90
,
and
150
.
The
cellular
immunity
of
the
patient
was
restored
,
and
infectious
complications
decreased
after
BMT
.
Later
the
patient
experienced
chronic
GVHD
,
and
he
died
on
day
post-transplantation
246
from
GVHD
.
BMT
without
conditioning
regimen
for
OS
is
feasible
,
but
there
must
be
a
megadose
cell
transplantation
,
and
appropriate
prophylactic
immunosuppressive
treatment
to
prevent
acute
GVHD
.
Diseases
Validation
Diseases presenting
"appropriate prophylactic immunosuppressive treatment"
symptom
omenn syndrome
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