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High prevalence of anaphylaxis in patients with systemic mastocytosis - a single-centre experience.
[cutaneous mastocytosis]
Systemic
mastocytosis
(
SM
)
is
a
clonal
mast
cells
disorder
characterized
by
the
proliferation
,
accumulation
and
activation
of
mast
cells
in
extracutaneous
tissues
.
The
clinical
picture
is
heterogeneous
and
may
range
from
asymptomatic
to
potentially
fatal
anaphylactic
reactions
due
to
excessive
mast
cell
mediator
release
.
The
aim
of
this
study
was
to
investigate
the
prevalence
and
trigger
factors
of
anaphylactic
reactions
among
adult
SM
patients
.
We
also
explored
the
clinical
spectrum
of
mast
cell
mediator-related
symptoms
in
patients
with
SM
.
T
his
descriptive
study
was
performed
among
84
consecutive
adult
(
≥
18
years
)
patients
those
were
diagnosed
with
SM
according
to
WHO
criteria
.
Sixty
-
six
of
the
patients
also
underwent
a
comprehensive
allergy
work-up
.
Sixty
of
84
patients
with
SM
(
71
%
)
had
bone
marrow
mast
cell
aggregates
and
fulfilled
the
major
criteria
for
SM
and
76
patients
(
91
%
)
had
indolent
disease
.
Simultaneous
occurrence
of
cutaneous
mastocytosis
was
observed
in
59
patients
(
70
%
)
.
Thirty
-
six
patients
(
43
%
)
had
had
at
least
one
episode
of
an
anaphylactic
reaction
.
The
clinical
courses
of
the
reactions
were
usually
severe
and
patients
often
presented
with
syncope
attacks
(
72
%
)
.
Most
patients
reacted
after
hymenoptera
venom
stings
(
19
/
36
;
53
%
)
.
In
39
%
(
14
/
36
)
,
a
clear
aetiology
could
not
be
determined
.
While
males
and
females
were
equally
frequent
among
the
patients
with
SM
,
anaphylaxis
patients
showed
a
male
predominance
(
61
%
)
.
Anaphylactic
reactions
occurred
more
frequently
in
patients
without
cutaneous
engagement
.
The
rate
of
allergy
sensitization
was
significantly
higher
in
SM
patients
with
anaphylaxis
as
compared
with
non-anaphylaxis
SM
patients
,
70
%
vs
.
23
%
,
respectively
(
P
=
0
.
0002
)
.
Anaphylaxis
is
more
prevalent
in
patients
with
SM
,
predominantly
in
patients
with
atopic
SM
.
Hymenoptera
venom-induced
and
idiopathic
anaphylaxis
were
the
most
frequent
elicitors
.
Our
findings
implicate
that
all
mastocytosis
patients
with
anaphylaxis
should
undergo
detailed
allergological
assessment
before
considering
treatment
and
preventive
measures
.
Diseases
Validation
Diseases presenting
"fulfilled the major criteria for sm and 76 patients"
symptom
cutaneous mastocytosis
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