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Multiparameter flow cytometry for the identification of the Waldenström's clone in IgM-MGUS and Waldenström's Macroglobulinemia: new criteria for differential diagnosis and risk stratification.
[waldenström macroglobulinemia]
Although
multiparameter
flow
cytometry
(
MFC
)
has
demonstrated
clinical
relevance
in
monoclonal
gammopathy
of
undetermined
significance
(
MGUS
)
/
myeloma
,
immunophenotypic
studies
on
the
full
spectrum
of
Waldenström
's
Macroglobulinemia
(
WM
)
remain
scanty
.
Herein
,
a
comprehensive
MFC
analysis
on
bone
marrow
samples
from
244
newly
diagnosed
patients
with
an
immunoglobulin
M
(
IgM
)
monoclonal
protein
was
performed
,
including
67
IgM-MGUS
,
77
smoldering
and
100
symptomatic
WM
.
Our
results
show
a
progressive
increase
on
the
number
and
light-chain-isotype-
positive
B-
cells
from
IgM-MGUS
to
smoldering
and
symptomatic
WM
(
P
<
.
001
)
,
with
only
1
%
of
IgM-MGUS
patients
showing
>
10
%
B
cells
or
100
%
light-chain-isotype-
positive
B-
cells
(
P
<
.
001
)
.
Complete
light-chain
restriction
of
the
B-
cell
compartment
was
an
independent
prognostic
factor
for
time-
to
progression
in
smoldering
WM
(
median
26
months
;
HR
:
19
.
8
,
P
=
0
.
001
)
and
overall
survival
in
symptomatic
WM
(
median
44
months
;
HR
:
2
.
6
,
P
=
0
.
004
)
.
The
progressive
accumulation
of
light-chain-isotype-
positive
B-
cells
accompanied
the
emergence
of
a
characteristic
Waldenstrom
's
phenotype
(
CD
22
(
+
dim
)
/
CD
2
5
+
/
CD
27
+
/
IgM
+
)
that
differed
from
other
B-NHL
by
negative
expression
of
CD
5
,
CD
10
,
CD
11
c
or
CD
103
.
In
contrast
to
myeloma
,
light-chain-isotype-
positive
plasma
cells
in
IgM
monoclonal
gammopathies
show
otherwise
normal
antigenic
expression
.
Our
results
highlight
the
potential
value
of
MFC
immunophenotyping
for
the
characterization
of
the
Waldenström
's
clone
,
as
well
as
for
the
differential
diagnosis
,
risk
of
progression
and
survival
in
WM
.
Diseases
Validation
Diseases presenting
"independent prognostic factor"
symptom
carcinoma of the gallbladder
cholangiocarcinoma
cutaneous mastocytosis
esophageal adenocarcinoma
esophageal carcinoma
esophageal squamous cell carcinoma
hodgkin lymphoma, classical
severe combined immunodeficiency
von hippel-lindau disease
waldenström macroglobulinemia
wiskott-aldrich syndrome
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