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Waldenström macroglobulinemia: 2013 update on diagnosis, risk stratification, and management.
[waldenström macroglobulinemia]
Waldenström
macroglobulinemia
(
WM
)
is
a
lymphoplasmacytic
lymphoma
with
immunoglobulin
M
(
IgM
)
monoclonal
protein
.
Clinical
features
include
anemia
,
thrombocytopenia
,
hepatosplenomegaly
,
and
lymphadenopathy
.
The
presence
of
IgM
monoclonal
protein
associated
with
≥
10
%
clonal
lymphoplasmacytic
cells
in
bone
marrow
confirms
the
diagnosis
.
Age
,
hemoglobin
level
,
platelet
count
,
β
2
microglobulin
,
and
monoclonal
IgM
concentrations
are
characteristics
required
for
prognosis
.
Not
all
patients
who
fulfill
WM
criteria
require
therapy
;
these
patients
can
be
observed
until
symptoms
develop
.
Rituximab-based
therapy
is
used
in
virtually
all
US
patients
with
WM
and
can
be
combined
with
alkylating
agent
or
purine
nucleoside
analog
(
or
both
)
.
The
preferred
Mayo
Clinic
nonstudy
therapeutic
induction
is
rituximab
,
cyclophosphamide
,
and
dexamethasone
.
Future
stem
cell
transplantation
should
be
considered
in
induction
therapy
selection
.
Bortezomib
,
thalidomide
,
everolimus
,
lenalidomide
,
and
bendamustine
have
all
been
shown
to
have
activity
in
WM
.
Given
WM
's
natural
history
,
reduction
of
complications
will
be
a
priority
for
future
treatment
trials
.
Diseases
Validation
Diseases presenting
"thrombocytopenia"
symptom
22q11.2 deletion syndrome
adrenomyeloneuropathy
dedifferentiated liposarcoma
erythropoietic protoporphyria
esophageal adenocarcinoma
heparin-induced thrombocytopenia
hodgkin lymphoma, classical
holt-oram syndrome
homocystinuria without methylmalonic aciduria
liposarcoma
malignant atrophic papulosis
monosomy 21
oculocutaneous albinism
scrub typhus
sneddon syndrome
typhoid
waldenström macroglobulinemia
wiskott-aldrich syndrome
This symptom has already been validated