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Systemic capillary leak syndrome.
[systemic capillary leak syndrome]
Systemic
capillary
leak
syndrome
is
a
rare
,
severe
disorder
with
a
high
mortality
rate
.
It
consists
of
the
shift
of
fluid
and
proteins
from
the
intravascular
to
the
extravascular
compartment
with
subsequent
hypovolemic
shock
.
We
describe
a
34
-
year
-old-woman
who
had
several
episodes
of
generalized
edema
that
evolved
to
hypovolemic
shock
.
During
the
acute
phase
,
laboratory
investigations
revealed
marked
hypoproteinemia
,
leukocytosis
,
and
high
levels
of
hematocrit
and
hemoglobin
.
A
paraprotein
IgG
kappa
chain
was
detected
.
Although
different
therapeutic
trials
were
used
,
the
patient
continued
to
have
similar
episodes
and
she
died
during
an
acute
episode
2
1
/
2
years
after
the
first
symptom
of
this
disorder
.
The
cause
of
systemic
capillary
leak
syndrome
is
unknown
.
The
presence
of
a
paraprotein
IgG
is
frequent
in
this
group
of
patients
.
Diseases
Validation
Diseases presenting
"and high levels of hematocrit and hemoglobin"
symptom
systemic capillary leak syndrome
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