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[Erdhei-Chester disease: report of one case].
[erdheim-chester disease]
We
report
a
76
-
year
-old
male
who
was
admitted
due
to
progressive
congestive
heart
failure
lasting
several
months
.
An
echocardiogram
showed
a
large
pericardial
effusion
with
early
signs
of
pericardial
tamponade
and
an
irregular
surface
suggestive
of
cancer
infiltration
.
The
patient
was
operated
,
creating
a
pericardial
window
and
draining
1
,
200
ml
of
a
brownish
yellow
fluid
with
abundant
cellularity
.
Pericardial
biopsy
showed
infiltration
by
CD
68
(
+
)
,
CD
1
a
(
-
)
and
S
100
(
-
)
cells
.
Twenty
-
eight
months
earlier
,
due
to
fatigue
,
dyspnea
,
and
a
non-
specific
inflammatory
process
,
an
enhanced-contrast-scan
showed
that
aorta
was
coated
with
a
hypodense
tissue
that
began
near
the
aortic
valve
and
extended
until
the
inferior
mesenteric
artery
,
with
stenosis
of
the
left
subclavian
,
celiac
axis
,
renal
and
upper
mesenteric
arteries
.
An
angioplasty
and
stent
placing
was
carried
out
in
the
last
two
arteries
.
Both
kidneys
had
the
appearance
of
"
hairy
kidneys
"
.
A
bone
scan
showed
increased
uptake
in
femurs
and
tibiae
and
X-
ray
examination
showed
osteosclerosis
in
metaphysis
and
diaphysis
.
The
diagnosis
of
Erdheim-
Chester
disease
(
non-
Langerhans-cell
histiocytosis
)
was
made
and
the
patient
was
treated
with
steroids
and
methotrexate
.
Diseases
Validation
Diseases presenting
"an irregular surface suggestive of cancer infiltration"
symptom
erdheim-chester disease
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