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Nodular lymphocyte-predominant Hodgkin lymphoma in children: clinical course, biology, and management.
[hodgkin lymphoma, classical]
Nodular
lymphocyte
predominant
Hodgkin
disease
(
NLPHL
)
differs
clearly
from
classical
Hodgkin
lymphoma
(
cHL
)
by
clinical
presentation
and
more
favorable
outcome
.
Patients
often
present
with
early
stage
IA
or
IIA
.
Extranodal
disease
and
B-
symptoms
are
uncommon
.
Histologically
,
NLPHL
is
characterized
by
the
presence
of
atypical
"
lymphocyte
predominant
cells
"
(
LP
cells
)
or
"
pop-corn
"
cells
in
a
non-neoplastic
and
reactionnal
nodular
background
of
small
mature
B-
lymphocytes
.
LP
cells
are
negative
for
CD
30
and
positive
for
CD
2
0
,
BCL
6
and
EMA
(
in
half
of
the
cases
)
.
FDG-PET
plays
an
important
role
in
evaluation
of
cHL
and
NLPHL
for
staging
,
therapy
assessment
and
relapse
.
Historically
,
patients
with
NLPHL
have
been
treated
like
patients
with
cHL
,
but
their
very
favorable
prognosis
and
the
risk
of
late
complications
of
chemotherapy
and
/
or
radiotherapy
have
led
to
a
de
-escalation
in
recent
years
.
Patients
with
early
stage
could
be
treated
by
surgical
adenectomy
alone
or
associated
with
not
intensive
chemotherapy
.
Currently
,
there
is
no
consensus
regarding
to
the
optimal
treatment
of
patients
with
advanced
stage
.
Rituximab
used
as
monotherapy
or
in
association
with
chemotherapy
has
achieved
complete
or
partial
responses
.
The
outcome
of
NLPHL
is
singular
by
the
frequent
occurrence
of
late
relapses
and
the
risk
of
transformation
into
aggressive
B
lymphoma
justifying
an
extended
follow-up
.
Further
prospective
studies
are
needed
to
optimize
treatment
of
these
advanced
and
recurrent
forms
.
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