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Pentostatin, cyclophosphamide and rituximab is a safe and effective treatment in patients with Waldenström's macroglobulinemia.
[waldenström macroglobulinemia]
Waldenström
's
macroglobulinemia
(
WM
)
is
an
indolent
lym
phomaand
is
responsive
to
therapy
regimens
containing
alkylating
agents
,
purine
analogs
and
rituximab
if
treatment
becomes
necessary
.
We
initiated
a
multicenter
phase
II
trial
to
determine
the
safety
and
efficacy
of
a
regimen
containing
pentostatin
,
cyclophosphamide
and
rituximab
(
PER
)
in
patients
with
WM
.
Between
May
2005
and
December
2010
,
25
patients
with
WM
were
included
in
the
study
.
Twenty
-
one
patients
received
PER
as
first
-line
therapy
.
In
these
patients
,
2
-
year
progression-free
survival
was
83
.
6
%
and
2
-
year
overall
survival
was
100
%
.
Thirteen
patients
(
52
%
)
received
R
maintenance
therapy
.
In
these
patients
,
the
2
-
year
progression-free
survival
was
91
.
67
%
and
2
-
year
overall
survival
was
100
%
.
We
have
provided
evidence
that
PER
is
a
safe
and
effective
regimen
for
WM
.
Although
R
maintenance
therapy
after
PER
seemed
to
induce
a
better
long
-term
outcome
,
this
study
was
not
powered
to
address
this
issue
.
Diseases
Validation
Diseases presenting
"first-line therapy"
symptom
allergic bronchopulmonary aspergillosis
coats disease
erdheim-chester disease
hodgkin lymphoma, classical
kabuki syndrome
waldenström macroglobulinemia
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