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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
"relative incidence"
symptom
hodgkin lymphoma, classical
inclusion body myositis
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