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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
.
Diseases
Validation
Diseases presenting
"fatigue"
symptom
benign recurrent intrahepatic cholestasis
cholangiocarcinoma
congenital adrenal hyperplasia
cowden syndrome
cutaneous mastocytosis
dedifferentiated liposarcoma
dracunculiasis
erdheim-chester disease
esophageal carcinoma
fabry disease
familial hypocalciuric hypercalcemia
familial mediterranean fever
hydrocephalus with stenosis of the aqueduct of sylvius
inclusion body myositis
kabuki syndrome
lymphangioleiomyomatosis
neuralgic amyotrophy
pleomorphic liposarcoma
systemic capillary leak syndrome
triple a syndrome
typhoid
von hippel-lindau disease
waldenström macroglobulinemia
x-linked adrenoleukodystrophy
This symptom has already been validated
