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[A rare cause of edema: sporadic lymphangioleiomyomatosis].
[lymphangioleiomyomatosis]
A
45
-
year
-old
woman
presented
with
marked
edema
of
both
lower
extremities
over
6
weeks
for
a
nephrological
work-up
;
she
had
gained
8
kg
of
body
weight
.
Voiding
was
asymptomatic
and
she
had
a
stable
diuresis
.
The
patient
took
oestrogens
for
contraception
over
10
years
.
Blood
pressure
was
normotensive
.
Serum-creatinine
was
0
.
8
mg
/
dl
;
a
slight
microalbuminuria
was
noted
.
Left
and
right
ventricular
systolic
function
were
normal
.
DIAGNOSTIC
FINDINGS
,
TREATMENT
AND
CLINICAL
COURSE
:
Computed
tomography
of
the
abdomen
revealed
a
hemodynamically
relevant
obstruction
of
the
venous
blood
flow
or
the
lymphatic
vessels
as
cause
for
the
edema
of
both
legs
.
Masses
of
lymphangioleiomyomas
located
around
the
v
.
cava
inferior
were
documented
.
Biopsy
of
the
masses
proved
a
massive
proliferation
of
smooth
muscle
cells
and
epitheloid
cells
with
an
immunohistochemically
typical
staining
.
Furthermore
,
CT
revealed
multiple
pulmonary
cysts
in
both
lungs
,
results
which
are
pathognomic
for
lymphangioleiomyomatosis
(
LAM
)
.
In
our
patient
,
the
structural
impairment
of
the
lungs
was
not
substantiated
clinically
,
i
.
e
.
she
had
only
slight
dyspnoe
during
exertion
.
By
mild
diuretic
treatment
with
HCT
and
fluid
control
,
a
moderate
regression
of
the
edema
was
achieved
.
At
present
,
the
mTOR
inhibitor
rapamycin
as
antiproliferative
treatment
is
considered
to
reduce
the
retroperitoneal
LAM
-related
masses
on
an
individual
basis
.
LAM
is
a
rare
genetically
determined
progressive
disease
occurring
frequently
in
women
in
childbearing
age
.
LAM
is
characterized
by
a
proliferation
of
smooth
muscle
cells
,
lymphangioma
,
renal
angiomyolipoma
,
pulmonary
cysts
and
progressive
destruction
of
lung
parenchyma
.
Refractory
edema
can
result
from
an
obstruction
of
the
venous
blood
and
lymphatic
flow
by
lymphangioma
masses
located
paracaval
,
which
was
the
impressive
and
first
clinical
feature
of
LAM
in
our
case
report
.
Diseases
Validation
Diseases presenting
"right ventricular"
symptom
22q11.2 deletion syndrome
fabry disease
lymphangioleiomyomatosis
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