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Indolent systemic mastocytosis: a case and review of the current available treatment options.
[cutaneous mastocytosis]
Mastocytosis
denotes
a
heterogeneous
group
of
disorders
characterized
by
abnormal
growth
and
accumulation
of
mast
cells
.
Cutaneous
mastocytosis
is
characterized
by
skin
-
limited
disease
and
is
the
most
common
subtype
.
Systemic
mastocytosis
has
extracutaneous
organ
involvement
with
variable
symptomatology
and
prognosis
.
Clinical
manifestations
are
secondary
to
mediator
release
or
direct
organ
infiltration
of
mast
cells
.
Current
available
treatment
for
systemic
mastocytosis
is
non-curative
with
conventional
symptom-directed
therapy
for
all
subtypes
.
Cytoreductive
agents
are
available
for
those
with
refractory
symptoms
or
extensive
extracutaneous
disease
.
To
date
,
clinical
responses
remain
mixed
and
systemic
mastocytosis
is
still
an
incurable
condition
.