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Primary mediastinal large B-cell lymphoma.
[hodgkin lymphoma, classical]
Primary
mediastinal
B-
cell
lymphoma
(
PMBCL
)
is
a
relatively
rare
lymphoma
subtype
affecting
mainly
young
adults
.
Its
molecular
signature
and
clinical
features
resemble
classical
Hodgkin
lymphoma
.
The
optimal
chemotherapy
for
this
lymphoma
subtype
has
not
been
established
.
The
addition
of
rituximab
to
anthracycline
based
chemotherapy
improved
response
rates
and
survival
.
Many
centers
use
R-CHOP
as
standard
treatment
,
but
the
role
of
the
intensified
regimens
and
consolidation
radiotherapy
has
to
be
clarified
.
Recent
data
coming
from
retrospective
analyses
and
an
ongoing
prospective
study
addressing
the
problem
of
consolidation
radiotherapy
will
help
to
better
identify
risk
groups
and
apply
risk-adapted
and
effective
treatment
strategies
.
The
latest
research
has
helped
to
understand
molecular
mechanisms
of
PMBCL
pathogenesis
and
indicated
targets
of
directed
therapy
for
the
future
.
