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[Systemic capillary leak syndrome presenting remarkable erythrocytosis].
[systemic capillary leak syndrome]
Systemic
capillary
leak
syndrome
(
SCLS
)
is
a
disorder
characterized
by
hypotension
,
edema
,
and
an
increased
hematocrit
(
Ht
)
due
to
sudden
leakage
of
plasma
into
the
extravascular
space
through
some
unknown
mechanism
,
in
which
monoclonal
gammopathy
is
observed
.
A
30
-
year
-old
man
visited
our
emergency
department
because
of
abdominal
pain
,
and
was
admitted
to
our
hematology
department
because
of
a
markedly
increased
hemoglobin
concentration
reaching
26
.
2
g
/
dl
.
The
polycythemia
was
thought
to
be
pseudo-
polycythemia
due
to
hemoconcentration
,
and
we
diagnosed
the
patient
as
having
SCLS
based
on
the
triad
of
increased
hematocrit
,
whole-body
edema
which
was
especially
marked
in
the
lower
extremities
,
and
monoclonal
gammopathy
.
The
patient
recovered
after
administration
of
extracellular
fluids
and
albumin
,
but
the
attacks
recurred
.
Prophylaxis
with
terbutaline
sulfate
,
theophylline
and
corticosteroid
reduced
the
frequency
of
severe
attacks
.
Because
there
is
possibility
that
patients
with
SCLS
may
be
admitted
to
hematology
departments
due
to
severe
erythrocytosis
,
we
report
this
case
to
increase
the
awareness
of
hematologists
that
SCLS
is
one
of
the
important
differential
diagnoses
of
erythrocytosis
.
Diseases
Validation
Diseases presenting
"sudden leakage of plasma into the extravascular space through some unknown mechanism"
symptom
systemic capillary leak syndrome
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