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Waldenström macroglobulinemia.
[waldenström macroglobulinemia]
Waldenström
macroglobulinemia
(
WM
)
is
an
IgM-secreting
B-
cell
lymphoproliferative
disorder
,
with
strong
familial
predisposition
.
MYD
88
L
265
P
and
CXCR
4
WHIM
mutations
are
common
in
WM
and
support
the
growth
and
survival
of
WM
cells
.
Clinical
manifestations
of
disease
are
related
to
both
tumor
cell
infiltration
and
paraprotein
production
.
Current
treatment
includes
monoclonal
antibodies
,
alkylating
agents
,
nucleoside
analogs
,
proteasome
inhibitors
,
immunomodulatory
drugs
,
and
signal
inhibitors
.
Short
-
and
long
-term
toxicities
should
be
weighed
in
treatment
decisions
with
use
of
these
agents
.
Elucidation
of
the
signaling
pathways
involved
in
WM
is
helping
to
advance
targeted
therapeutics
for
WM
and
includes
efforts
directed
at
MYD
88
and
CXCR
4
signaling
.
Diseases
Validation
Diseases presenting
"with strong familial predisposition"
symptom
waldenström macroglobulinemia
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