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Systemic capillary leak syndrome preceding plasma cell leukaemia.
[systemic capillary leak syndrome]
We
report
a
patient
with
plasma
cell
leukaemia
with
systemic
capillary
leak
syndrome
,
a
rare
disorder
often
associated
with
monoclonal
gammopathy
.
In
this
patient
,
the
manifestation
of
capillary
leak
syndrome
antedated
the
diagnosis
of
plasma
cell
leukaemia
by
5
-
6
months
.
During
that
time
,
he
was
repeatedly
admitted
to
the
hospital
with
weight
gain
,
congestive
cardiac
failure
,
cough
and
anasarca
in
the
presence
of
normal
renal
function
,
liver
function
and
normal
echocardiography
.
On
presentation
,
a
serum
protein
electrophoresis
showed
monoclonal
IgG
;
the
blood
smear
showed
60
%
plasma
cells
with
a
total
count
of
4
.
4
x
10
(
9
)
/
l
.
A
bone
marrow
aspirate
showed
replacement
of
the
normal
marrow
by
sheets
of
immature
plasma
cells
.
His
systemic
capillary
leak
syndrome
initially
responded
to
decongestive
therapy
with
terbutaline
and
aminophylline
but
later
on
he
became
refractory
to
them
and
responded
to
vincristine
,
doxorubicin
and
dexamethasone
(
VAD
)
combination
therapy
only
transiently
.
Danocrine
and
pentoxifylline
,
added
during
VAD
chemotherapy
,
did
not
produce
a
durable
response
in
capillary
leak
syndrome
,
which
finally
responded
to
autologous
peripheral
blood
stem
cell
transplantation
(
PBSCT
)
.
After
PBSCT
,
he
remained
free
of
capillary
leak
for
10
months
without
terbutaline
,
pentoxifylline
corticosteroids
,
aminophylline
or
danocrine
.
His
disease
relapsed
without
recurrence
of
the
capillary
leak
.
He
died
15
months
after
PBSCT
and
20
months
after
the
diagnosis
of
plasma
cell
leukaemia
.
Diseases
Validation
Diseases presenting
"liver function"
symptom
benign recurrent intrahepatic cholestasis
canavan disease
carcinoma of the gallbladder
cutaneous mastocytosis
erythropoietic protoporphyria
heparin-induced thrombocytopenia
krabbe disease
primary hyperoxaluria type 1
pyomyositis
scrub typhus
systemic capillary leak syndrome
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