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Lymphocyte-predominant Hodgkin lymphoma: what is the optimal treatment?
[hodgkin lymphoma, classical]
Nodular
lymphocyte-predominant
Hodgkin
lymphoma
(
NLPHL
)
is
a
unique
diagnostic
entity
,
with
only
∼
500
new
cases
in
the
United
States
per
year
with
a
similar
infrequent
incidence
worldwide
.
NLPHL
also
has
distinctive
pathobiology
and
clinical
characteristics
compared
with
the
more
common
classical
Hodgkin
lymphoma
(
cHL
)
,
including
CD
2
0
positivity
of
the
pathognomic
lymphocytic
and
histiocytic
cells
and
an
overall
more
indolent
course
with
a
higher
likelihood
of
delayed
relapses
.
Given
the
limited
numbers
of
prospective
NLPHL
-focused
trials
,
management
algorithms
historically
have
typically
been
centered
on
retrospective
data
with
guidelines
often
adopted
from
cHL
and
indolent
B-
cell
lymphoma
treatment
approaches
.
Key
recent
publications
have
delineated
that
NLPHL
has
a
higher
level
of
pathological
overlap
with
cHL
and
the
aggressive
B-
cell
lymphomas
than
with
indolent
B-
cell
lymphomas
.
Over
the
past
decade
,
there
has
been
a
series
of
NLPHL
publications
that
evaluated
the
role
of
rituximab
in
the
frontline
and
relapsed
setting
,
described
the
relative
incidence
of
transformation
to
aggressive
B-
cell
lymphomas
,
weighed
the
benefit
of
addition
of
chemotherapy
to
radiation
treatment
for
patients
with
early
-
stage
disease
,
considered
what
should
be
the
preferred
chemotherapy
regimen
for
advanced
-
stage
disease
,
and
even
assessed
the
potential
role
of
autologous
stem
cell
transplantation
for
the
management
of
relapsed
disease
.
General
themes
within
the
consensus
guidelines
include
the
role
for
radiation
treatment
as
a
monotherapy
for
early
-
stage
disease
,
the
value
of
large
B-
cell
lymphoma
-directed
regimens
for
transformed
disease
,
the
utility
of
rituximab
for
treatment
of
relapsed
disease
,
and
,
in
the
pediatric
setting
,
the
role
of
surgical
management
alone
for
patients
with
early
-
stage
disease
.
Diseases
Validation
Diseases presenting
"hodgkin lymphoma"
symptom
esophageal adenocarcinoma
hodgkin lymphoma, classical
monosomy 21
primary effusion lymphoma
severe combined immunodeficiency
systemic capillary leak syndrome
waldenström macroglobulinemia
This symptom has already been validated