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Venom immunotherapy in patients with mastocytosis and hymenoptera venom anaphylaxis.
[cutaneous mastocytosis]
Systemic
mastocytosis
(
SM
)
is
typically
suspected
in
patients
with
cutaneous
mastocytosis
(
CM
)
.
In
recent
years
,
the
presence
of
clonal
mast
cells
(
MCs
)
in
a
subset
of
patients
with
systemic
symptoms
associated
with
MC
activation
in
the
absence
of
CM
has
been
reported
and
termed
monoclonal
MC
activation
syndromes
or
clonal
systemic
MC
activation
syndromes
.
In
these
cases
,
bone
marrow
(
BM
)
MC
numbers
are
usually
lower
than
in
SM
with
CM
,
there
are
no
detectable
BM
MC
aggregates
,
and
serum
baseline
tryptase
is
often
<
20
µg
/
l
;
thus
,
diagnosis
of
SM
in
these
patients
should
be
based
on
careful
evaluation
of
other
minor
WHO
criteria
for
SM
in
reference
centers
,
where
highly
sensitive
techniques
for
immunophenotypic
analysis
and
investigation
of
KIT
mutations
on
fluorescence-activated
cell
sorter-purified
BM
MCs
are
routinely
performed
.
The
prevalence
of
hymenoptera
venom
anaphylaxis
(
HVA
)
among
SM
patients
is
higher
than
among
the
normal
population
and
it
has
been
reported
to
be
approximately
5
%
.
In
SM
patients
with
IgE-mediated
HVA
,
venom
immunotherapy
is
safe
and
effective
and
it
should
be
prescribed
lifelong
.
Severe
adverse
reactions
to
hymenoptera
stings
or
venom
immunotherapy
have
been
associated
with
increased
serum
baseline
tryptase
;
however
,
presence
of
clonal
MC
has
not
been
ruled
out
in
most
reports
and
thus
both
SM
and
clonal
MC
activation
syndrome
might
be
underdiagnosed
in
such
patients
.
In
fact
,
clonal
BM
MC
appears
to
be
a
relevant
risk
factor
for
both
HVA
and
severe
reactions
to
venom
immunotherapy
,
while
the
increase
in
serum
baseline
tryptase
by
itself
should
be
considered
as
a
powerful
surrogate
marker
for
anaphylaxis
.
The
Spanish
Network
on
Mastocytosis
has
developed
a
scoring
system
based
on
patient
gender
,
the
clinical
symptoms
observed
during
anaphylaxis
and
serum
baseline
tryptase
to
predict
for
the
presence
of
both
MC
clonality
and
SM
among
individuals
who
suffer
from
anaphylaxis
.
Diseases
Validation
Diseases presenting
"mastocytosis"
symptom
allergic bronchopulmonary aspergillosis
cutaneous mastocytosis
epidermolysis bullosa simplex
This symptom has already been validated