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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
"edema"
symptom
acute rheumatic fever
adrenomyeloneuropathy
aniridia
cadasil
canavan disease
classical phenylketonuria
coats disease
congenital toxoplasmosis
cushing syndrome
cutaneous mastocytosis
erythropoietic protoporphyria
esophageal carcinoma
fabry disease
familial mediterranean fever
focal myositis
harlequin ichthyosis
homocystinuria without methylmalonic aciduria
inclusion body myositis
lamellar ichthyosis
liposarcoma
lymphangioleiomyomatosis
malignant atrophic papulosis
pleomorphic liposarcoma
systemic capillary leak syndrome
trochlear dysplasia
von hippel-lindau disease
waldenström macroglobulinemia
This symptom has already been validated