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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
·
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
.
