Rare Diseases Symptoms Automatic Extraction
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Extramedullary Waldenström macroglobulinemia.
[waldenström macroglobulinemia]
Disease
assessment
in
Waldenstrom
Macroglobulinemia
(
WM
)
is
dependent
on
the
percent
involvement
of
B-
cell
neoplasm
in
the
bone
marrow
and
IgM
paraprotein
in
the
serum
.
A
subset
of
patients
also
demonstrates
extramedullary
involvement
,
which
is
infrequently
examined
.
The
role
of
extramedullary
involvement
in
the
diagnosis
and
prognosis
of
WM
is
poorly
understood
.
The
purpose
of
this
study
is
to
report
the
characteristics
of
WM
patients
with
extramedullary
disease
(
EMD
)
.
Nine
hundred
and
eight
-
five
patients
with
WM
were
evaluated
at
one
academic
center
and
the
presence
of
EMD
was
assessed
in
these
patients
.
Forty
-
three
(
4
.
4
%
)
patients
were
identified
to
have
EMD
.
Nine
(
21
%
)
patients
presented
with
involvement
at
WM
diagnosis
,
while
34
(
79
%
)
developed
EMD
post-therapy
for
WM
.
Most
frequent
EMD
sites
involved
were
pulmonary
(
30
%
)
,
soft
tissue
(
21
%
)
,
cerebrospinal
fluid
(
23
%
)
,
renal
(
8
%
)
,
and
bone
(
9
%
)
.
The
median
overall
survival
at
10
years
was
79
%
(
95
%
CI
:
57
-
90
%
)
.
This
is
the
first
study
to
describe
the
clinical
characteristics
,
response
and
overall
survival
in
patients
with
extramedullary
WM
.
Further
studies
to
define
the
molecular
characteristics
of
this
entity
and
mechanisms
of
its
development
are
warranted
.