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Erdheim-Chester disease with prominent pericardial effusion: cytologic findings and review of the literature.
[erdheim-chester disease]
Erdheim-
Chester
disease
(
ECD
)
is
a
rare
,
non-
Langerhans
form
of
histiocytosis
of
unknown
origin
with
distinct
clinicopathologic
and
radiographic
features
.
Reports
detailing
the
cytology
of
ECD
are
rare
.
We
describe
a
case
of
ECD
with
pericardial
effusion
.
Cytologic
examination
revealed
a
hypercellular
specimen
composed
of
clusters
and
singly
dispersed
foamy
macrophages
with
round
nuclei
and
inconspicuous
nucleoli
,
admixed
with
lymphocytes
,
eosinophils
,
and
Touton-
type
multinucleated
giant
cells
.
Immunostains
for
CD
68
were
strongly
positive
in
the
foamy
macrophages
while
S
100
and
CD
1
a
were
negative
.
The
presence
of
foamy
histiocytes
,
multinucleated
giant
cells
,
lymphocytes
and
eosinophils
are
also
features
of
other
systemic
histiocytic
disorders
,
including
Langerhans
cell
histiocytosis
(
LCH
)
,
Rosai-
Dorfman
disease
(
RDD
)
and
sarcoidosis
.
To
the
best
of
out
knowledge
,
this
is
the
first
report
describing
the
cytological
features
of
ECD
in
a
pericardial
effusion
.