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Impact of anakinra treatment on cytokine and lymphocytes/ monocytes profile of an Erdheim-Chester patient.
[erdheim-chester disease]
Erdheim-
Chester
disease
(
ECD
)
is
a
rare
non-
Langerhans
cells
histiocytosis
associated
with
intense
immune
activation
.
In
our
clinical
center
,
an
ECD
patient
was
treated
with
anakinra
,
IL
1
RA
(
interleukin
1
receptor
antagonist
)
,
resulting
in
clinical
improvement
and
major
decrease
of
pathological
fatigue
.
The
aim
of
the
study
was
to
evaluate
changes
in
cytokine
profile
and
shift
of
immune
cells
estimated
by
flow
cytometric
analysis
of
ECD
patient
before
,
during
initial
stages
of
anakinra
treatment
as
well
as
after
treatment
ceased
in
comparison
to
healthy
donors
.
Singleplex
reactions
of
19
individual
cytokines
from
serum
of
ECD
patient
were
measured
by
FACS
array
.
Flow
cytometric
analyses
were
performed
on
peripheral
blood
cells
.
The
most
striking
result
is
substantial
decrease
of
IL
6
immediately
after
anakinra
treatment
started
suggesting
a
major
role
of
IL
1
pathway
in
ECD
pathophysiology
.
As
for
flow
cytometric
analysis
,
increased
number
of
CD
16
+
monocytes
before
treatment
is
a
new
finding
.
Our
results
suggest
that
IL
6
may
be
a
marker
of
early
treatment
response
of
ECD
patients
treated
with
anakinra
.
