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Long-term outcome of monoclonal (type 1) cryoglobulinemia.
[waldenström macroglobulinemia]
The
aim
of
this
study
is
to
investigate
long
-term
outcome
of
symptomatic
type
1
cryoglobulinemia
(
CG
)
and
its
determinants
.
Retrospective
cohort
study
was
conducted
in
two
French
University
Hospitals
.
Patients
with
type
1
CG
were
identified
using
laboratory
databases
.
Inclusion
criterion
was
the
presence
of
persistent
symptoms
of
CG
.
Among
227
screened
patients
,
36
were
included
.
Skin
or
vasomotor
symptoms
were
the
most
frequent
features
(
75
%
)
.
Nephropathy
and
neuropathy
occurred
in
30
%
and
47
%
of
cases
,
respectively
.
The
underlying
B
cell
disease
(
BCD
)
was
a
nonmalignant
monoclonal
gammopathy
(
NMMG
)
in
13
(
36
%
)
and
a
hematologic
malignancy
(
HM
)
in
23
(
64
%
;
Waldenstrom
macroglobulinemia
(
WM
)
in
12
,
low
-grade
non-
Hodgkin
lymphoma
(
NHL
)
in
6
,
multiple
myeloma
(
MM
)
in
4
,
and
chronic
lymphocytic
leukemia
in
1
patient
.
Severe
manifestations
affected
half
the
patients
and
were
more
frequent
with
IgG
(
82
vs
.
30
%
(
P
=
0
.
006
)
)
.
At
last
follow-up
,
64
%
of
patients
had
suffered
no
hematologic
manifestation
.
Potent
chemotherapeutic
regimens
were
mainly
used
in
HM
.
For
patients
with
NMMG
,
WM
,
or
NHL
,
fludarabine
or
rituximab-based
regimens
appeared
to
yield
better
responses
.
Five
-
year
survival
rate
was
82
%
.
In
multivariate
analysis
,
mortality
was
significantly
higher
in
older
patients
(
HR
:
1
.
17
per
year
[
95
%
CI
:
1
.
06
-
1
.
28
]
,
P
=
0
.
001
)
and
those
with
nephropathy
(
HR
:
8
.
9
[
95
%
CI
:
1
.
9
-
43
]
,
P
=
0
.
006
)
.
Kidney
disease
,
infections
,
Richter
's
transformation
,
and
second
malignancies
were
important
sources
of
morbi-mortality
.
Despite
its
limitations
,
this
series
provide
novel
information
regarding
type
1
CG
.
Further
studies
are
needed
to
improve
its
management
.
To
date
,
therapeutic
strategy
should
be
tailored
according
to
patient
's
characteristics
(
age
,
comorbidities
,
underlying
BCD
)
,
and
therapeutic
target
.
Diseases
Validation
Diseases presenting
"low-grade non-hodgkin"
symptom
waldenström macroglobulinemia
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