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New insights into the pathogenesis, diagnosis, and management of mastocytosis.
[cutaneous mastocytosis]
This
review
describes
developments
in
understanding
normal
mast
cell
function
and
genetic
changes
that
predispose
to
malignant
transformation
to
mastocytosis
.
Most
mastocytosis
cases
are
associated
with
somatically
acquired
activating
mutations
in
the
KIT
receptor
.
The
role
these
mutations
play
in
the
development
of
mastocytosis
is
discussed
.
Mastocytosis
is
classified
into
cutaneous
mastocytosis
and
systemic
mastocytosis
.
The
classification
of
mastocytosis
and
clinical
presentation
of
each
variant
is
detailed
in
this
report
.
Progress
has
been
made
in
developing
drugs
that
target
the
wild-
type
and
mutated
KIT
receptor
,
and
these
and
other
new
therapeutic
strategies
for
the
treatment
of
mastocytosis
are
reviewed
.