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Selective clinical and immune response of the oligoclonal autoreactive T cells in Omenn patients after cyclosporin A treatment.
[omenn syndrome]
The
immunological
hallmark
of
Omenn
syndrome
(
OS
)
is
the
expansion
and
activation
of
an
oligoclonal
population
of
autoreactive
T
cells
.
These
cells
should
be
controlled
rapidly
by
immunosuppressive
agents
,
such
as
cyclosporin
A
(
CsA
)
,
to
avoid
tissue
infiltration
and
to
improve
the
general
outcome
of
the
patients
.
Here
we
studied
the
clinical
and
the
immune
response
to
CsA
in
two
Omenn
patients
and
also
examined
the
gene
expression
profile
associated
with
good
clinical
response
to
such
therapy
.
T
cell
receptor
diversity
was
studied
in
cells
obtained
from
OS
patients
during
CsA
therapy
.
Characterization
of
gene
expression
in
these
cells
was
carried
out
by
using
the
TaqMan
low
-density
array
.
One
patient
showed
complete
resolution
of
his
symptoms
after
CsA
therapy
.
The
other
patient
showed
selective
response
of
his
oligoclonal
T
cell
population
and
combination
therapy
was
required
to
control
his
symptoms
.
Transcriptional
profile
associated
with
good
clinical
response
to
CsA
therapy
revealed
significant
changes
in
26
ยท
6
%
of
the
tested
genes
when
compared
with
the
transcriptional
profile
of
the
cells
before
treatment
.
Different
clinical
response
to
CsA
in
two
OS
patients
is
correlated
with
their
immunological
response
.
Varying
clonal
expansions
in
OS
patients
can
cause
autoimmune
features
and
can
respond
differently
to
immunosuppressive
therapy
;
therefore
,
additional
treatment
is
sometimes
indicated
.
CsA
for
OS
patients
causes
regulation
of
genes
that
are
involved
closely
with
self-tolerance
and
autoimmunity
.
Diseases
Validation
Diseases presenting
"complete resolution"
symptom
coats disease
cutaneous mastocytosis
focal myositis
homocystinuria without methylmalonic aciduria
malignant atrophic papulosis
oculocutaneous albinism
omenn syndrome
proteus syndrome
sneddon syndrome
systemic capillary leak syndrome
thoracic outlet syndrome
wiskott-aldrich syndrome
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