Rare Diseases Symptoms Automatic Extraction
Home
A random Abstract
Our Project
Our Team
Pathological consequence of misguided dendritic cell differentiation in histiocytic diseases.
[erdheim-chester disease]
Histiocytic
disorders
represent
a
group
of
complex
pathologies
characterized
by
the
accumulation
of
histiocytes
,
an
old
term
for
tissue-resident
macrophages
and
dendritic
cells
.
Langerhans
cell
histiocytosis
is
the
most
frequent
of
histiocytosis
in
humans
and
has
been
thought
to
arise
from
the
abnormal
accumulation
of
epidermal
dendritic
cells
called
Langerhans
cells
.
In
this
chapter
,
we
discuss
the
origin
and
differentiation
of
Langerhans
cells
and
dendritic
cells
and
present
accumulated
evidence
that
suggests
that
Langerhans
cell
histiocytosis
does
not
result
from
abnormal
Langerhans
cell
homeostasis
but
rather
is
a
consequence
of
misguided
differentiation
programs
of
myeloid
dendritic
cell
precursors
.
We
propose
reclassification
of
Langerhans
cell
histiocytosis
,
juvenile
xanthogranuloma
,
and
Erdheim-
Chester
disease
as
inflammatory
myeloid
neoplasias
.
