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Separate episodes of capillary leak syndrome and pulmonary hypertension after adjuvant gemcitabine and three years later after nab-paclitaxel for metastatic disease.
[systemic capillary leak syndrome]
Systemic
capillary
leak
syndrome
is
a
rare
disease
with
a
high
mortality
rate
.
This
syndrome
is
characterised
by
generalised
edema
,
hypotension
,
hemoconcentration
,
and
hypoproteinemia
.
The
cause
is
the
sudden
onset
of
capillary
hyperpermeability
with
extravasations
of
plasma
from
the
intravascular
to
the
extravascular
compartment
.
We
present
the
case
of
a
patient
who
experienced
two
episodes
of
systemic
capillary
leak
syndrome
and
pulmonary
hypertension
;
the
first
after
gemcitabine
in
an
adjuvant
setting
and
the
second
three
years
later
after
treatment
with
nab
-paclitaxel
for
metastatic
disease
.
A
65
-
year
-old
patient
underwent
a
pancreatectomy
in
January
2010
for
ductal
carcinoma
(
pT
3
N
0
M
0
,
stage
IIa
)
,
followed
by
adjuvant
chemotherapy
.
Seven
days
after
the
last
cycle
,
she
developed
dyspnea
associated
with
orthopnea
and
cough
.
A
transthoracic
cardiac
ecocolordoppler
was
performed
,
with
evidence
of
pulmonary
hypertension
(
58
mmHg
)
.
Blood
tests
showed
an
increase
in
creatinine
,
pro-
BNP
and
D-Dimer
.
She
began
high
-dose
diuretic
therapy
combined
with
cortisone
.
After
a
month
,
the
patient
was
eupneic
and
the
anasarca
had
resolved
.
We
decided
gradually
to
reduce
the
steroid
and
diuretic
therapy
.
After
ten
days
of
the
reduction
,
the
patient
began
to
re
-present
the
same
symptoms
after
treatment
with
gemcitabine
.
Corticosteroid
therapy
was
restored
with
rapid
clinical
benefit
and
decreased
pro-
BNP
after
a
week
of
treatment
.
After
two
years
,
the
disease
returned
.
As
a
first
line
treatment
,
it
was
decided
to
use
nab
-paclitaxel
100
mg
/
m
2
weekly
.
After
two
doses
,
followed
by
approximately
14
days
of
treatment
,
the
patient
developed
acute
respiratory
distress
syndrome
.
The
clinical
suspicion
was
a
relapse
of
capillary
leak
syndrome
and
treatment
with
a
high
-dose
diuretic
(
furosemide
250
mg
daily
)
was
started
combined
with
cortisone
(
40
mg
methylprednisolone
)
.
The
patient
showed
a
progressive
clinical
benefit
.
In
patients
treated
with
gemcitabine
and
nab
-paclitaxel
who
experience
a
sudden
onset
of
diffuse
edema
with
respiratory
distress
,
capillary
leak
syndrome
should
be
suspected
.
Immediate
treatment
with
corticosteroids
may
be
life-saving
.
Diseases
Validation
Diseases presenting
"systemic capillary leak syndrome"
symptom
systemic capillary leak syndrome
This symptom has already been validated