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Waldenström macroglobulinemia: clinical and immunological aspects, natural history, cell of origin, and emerging mouse models.
[waldenström macroglobulinemia]
Waldenström
macroglobulinemia
(
WM
)
is
a
rare
and
currently
incurable
neoplasm
of
IgM-expressing
B-
lymphocytes
that
is
characterized
by
the
occurrence
of
a
monoclonal
IgM
(
mIgM
)
paraprotein
in
blood
serum
and
the
infiltration
of
the
hematopoietic
bone
marrow
with
malignant
lymphoplasmacytic
cells
.
The
symptoms
of
patients
with
WM
can
be
attributed
to
the
extent
and
tissue
sites
of
tumor
cell
infiltration
and
the
magnitude
and
immunological
specificity
of
the
paraprotein
.
WM
presents
fascinating
clues
on
neoplastic
B-
cell
development
,
including
the
recent
discovery
of
a
specific
gain-of-function
mutation
in
the
MYD
88
adapter
protein
.
This
not
only
provides
an
intriguing
link
to
new
findings
that
natural
effector
IgM
(
+
)
IgD
(
+
)
memory
B-
cells
are
dependent
on
MYD
88
signaling
,
but
also
supports
the
hypothesis
that
WM
derives
from
primitive
,
innate-like
B-
cells
,
such
as
marginal
zone
and
B
1
B-
cells
.
Following
a
brief
review
of
the
clinical
aspects
and
natural
history
of
WM
,
this
review
discusses
the
thorny
issue
of
WM
's
cell
of
origin
in
greater
depth
.
Also
included
are
emerging
,
genetically
engineered
mouse
models
of
human
WM
that
may
enhance
our
understanding
of
the
biologic
and
genetic
underpinnings
of
the
disease
and
facilitate
the
design
and
testing
of
new
approaches
to
treat
and
prevent
WM
more
effectively
.
Diseases
Validation
Diseases presenting
"neoplasm"
symptom
adrenal incidentaloma
alexander disease
aromatase deficiency
carcinoma of the gallbladder
cholangiocarcinoma
cowden syndrome
cushing syndrome
cutaneous mastocytosis
dedifferentiated liposarcoma
dentin dysplasia
erdheim-chester disease
esophageal adenocarcinoma
esophageal carcinoma
familial hypocalciuric hypercalcemia
focal myositis
hodgkin lymphoma, classical
hydrocephalus with stenosis of the aqueduct of sylvius
junctional epidermolysis bullosa
kabuki syndrome
liposarcoma
lymphangioleiomyomatosis
pleomorphic liposarcoma
primary effusion lymphoma
proteus syndrome
pyomyositis
von hippel-lindau disease
waldenström macroglobulinemia
well-differentiated liposarcoma
werner syndrome
wolf-hirschhorn syndrome
This symptom has already been validated
