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A random Abstract
Our Project
Our Team
Langerhans cell histiocytosis and Erdheim-Chester disease.
[erdheim-chester disease]
To
provide
an
updated
overview
of
the
pathogenesis
and
treatment
of
Langerhans
cell
histiocytosis
(
LCH
)
and
Erdheim-
Chester
disease
(
ECD
)
.
There
is
ongoing
debate
as
to
the
exact
pathogenesis
of
these
disorders
and
their
classification
as
reactive
versus
neoplastic
.
Proinflammatory
cytokines
are
known
to
play
a
role
in
both
LCH
and
ECD
and
strengthen
the
hypothesis
that
,
at
least
in
part
,
they
are
disorders
of
immune
dysregulation
.
The
recent
discovery
of
activating
mutations
in
the
proto-oncogene
BRAF
in
a
subset
of
LCH
patients
suggests
that
LCH
is
in
fact
a
neoplastic
disorder
.
Understanding
of
the
mechanisms
that
promote
proliferation
and
migration
of
histiocytes
has
led
researchers
to
explore
targeted
immune-modulatory
therapies
for
ECD
.
Similarly
for
LCH
,
alternative
chemotherapeutic
agents
and
reduced-intensity
hematopoietic
stem
cell
transplant
are
being
evaluated
for
refractory
disease
.
More
research
is
needed
to
better
understand
the
cause
of
these
disorders
and
may
help
in
identifying
new
targeted
therapies
,
particularly
for
patients
with
refractory
or
relapsed
disease
.
Multinational
trials
are
ongoing
for
LCH
and
are
urgently
needed
for
ECD
.
