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[Shock, hemoconcentration, and generalized edema in a 47-year-old man].
[systemic capillary leak syndrome]
We
report
the
case
of
a
47
-
year
-old
man
who
was
admitted
because
of
syncope
.
Upon
hospital
admission
,
he
rapidly
developed
circulatory
shock
with
generalized
edema
and
a
severe
hemoconcentration
with
a
hematocrit
of
70
%
.
The
condition
was
stabilized
with
infusion
of
17
l
of
cristalloid
fluids
over
a
period
of
24
h
.
After
ruling
out
possible
secondary
causes
,
the
diagnosis
of
a
systemic
capillary
leak
syndrome
--a
severe
transient
endothelial
barrier
dysfunction
of
unknown
origin--was
made
.
A
triad
of
hypotension
,
hemoconcentration
(
hematocrit
>
60
%
)
and
macromolecular
extravasation
is
the
typical
finding
;
furthermore
,
a
strong
association
with
monoclonal
gammopathy
of
unknown
significance
(
MGUS
)
is
described
.
Diseases
Validation
Diseases presenting
"hypotension"
symptom
achondroplasia
adrenal incidentaloma
congenital adrenal hyperplasia
congenital diaphragmatic hernia
cutaneous mastocytosis
fabry disease
familial mediterranean fever
primary effusion lymphoma
scrub typhus
systemic capillary leak syndrome
thoracic outlet syndrome
typhoid
This symptom has already been validated
