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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
"dyspnea"
symptom
acute rheumatic fever
allergic bronchopulmonary aspergillosis
carcinoma of the gallbladder
congenital diaphragmatic hernia
cushing syndrome
cutaneous mastocytosis
dedifferentiated liposarcoma
erdheim-chester disease
esophageal carcinoma
fabry disease
familial mediterranean fever
inclusion body myositis
liposarcoma
lymphangioleiomyomatosis
neuralgic amyotrophy
pleomorphic liposarcoma
primary effusion lymphoma
scrub typhus
systemic capillary leak syndrome
thoracic outlet syndrome
well-differentiated liposarcoma
This symptom has already been validated