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Lymphatic endothelial differentiation in pulmonary lymphangioleiomyomatosis cells.
[lymphangioleiomyomatosis]
Pulmonary
lymphangioleiomyomatosis
(
LAM
)
is
a
rare
,
low
-grade
neoplasm
affecting
almost
exclusively
women
of
childbearing
age
.
LAM
belongs
to
the
family
of
perivascular
epithelioid
cell
tumors
,
characterized
by
spindle
and
epithelioid
cells
with
smooth
muscle
and
melanocytic
differentiation
.
LAM
cells
infiltrate
the
lungs
,
producing
multiple
,
bilateral
lesions
rich
in
lymphatic
channels
and
forming
cysts
,
leading
to
respiratory
insufficiency
.
Here
we
used
antibodies
against
four
lymphatic
endothelial
markers-
podoplanin
(
detected
by
D
2
-
40
)
,
prospero
homeobox
1
(
PROX
1
)
,
vascular
endothelial
growth
factor
receptor
3
(
VEGFR-
3
)
,
and
lymphatic
vessel
endothelial
hyaluronan
receptor
1
(
LYVE
1
)
-
to
determine
whether
LAM
cells
show
lymphatic
differentiation
.
Twelve
of
12
diagnostic
biopsy
specimens
(
early
-
stage
LAM
)
and
19
of
19
explants
(
late
-
stage
LAM
)
showed
immunopositivity
for
D
2
-
40
in
most
neoplastic
cells
.
PROX
1
,
VEGFR-
3
,
and
LYVE
1
immunoreactivity
varied
from
scarce
in
the
early
stage
to
abundant
in
the
late
stage
.
Lymphatic
endothelial
,
smooth
muscle
,
and
melanocytic
markers
were
partially
co
-
localized
.
These
findings
indicate
that
lymphatic
endothelial
differentiation
is
a
feature
of
LAM
and
provide
evidence
of
a
previously
unidentified
third
lineage
of
differentiation
in
this
neoplasm
.
This
study
has
implications
for
the
histological
diagnosis
of
LAM
,
the
origin
of
the
neoplastic
cells
,
and
potential
future
treatment
with
drugs
targeting
lymphangiogenesis
.
Diseases
Validation
Diseases presenting
"exclusively women"
symptom
lymphangioleiomyomatosis
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