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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
.
Diseases
Validation
Diseases presenting
"first report"
symptom
achondroplasia
alexander disease
aniridia
cadasil
canavan disease
child syndrome
cohen syndrome
congenital toxoplasmosis
cowden syndrome
cushing syndrome
cutaneous mastocytosis
cystinuria
dedifferentiated liposarcoma
dentinogenesis imperfecta
dracunculiasis
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
erdheim-chester disease
esophageal squamous cell carcinoma
fabry disease
familial mediterranean fever
focal myositis
harlequin ichthyosis
hirschsprung disease
hodgkin lymphoma, classical
holt-oram syndrome
homocystinuria without methylmalonic aciduria
inclusion body myositis
junctional epidermolysis bullosa
kabuki syndrome
kindler syndrome
krabbe disease
lamellar ichthyosis
liposarcoma
lymphangioleiomyomatosis
monosomy 21
neonatal adrenoleukodystrophy
neuralgic amyotrophy
oculocutaneous albinism
oligodontia
omenn syndrome
pendred syndrome
pleomorphic liposarcoma
primary hyperoxaluria type 1
pyomyositis
pyruvate dehydrogenase deficiency
scrub typhus
severe combined immunodeficiency
sneddon syndrome
triple a syndrome
typhoid
waldenström macroglobulinemia
werner syndrome
wiskott-aldrich syndrome
x-linked adrenoleukodystrophy
zellweger syndrome
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