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Clinical impact of pregnancy in mastocytosis: a study of the Spanish Network on Mastocytosis (REMA) in 45 cases.
[cutaneous mastocytosis]
The
impact
of
pregnancy
on
mast
cell
(
MC
)
-
related
symptoms
and
newborn
outcome
in
women
with
mastocytosis
is
not
well
described
.
We
report
a
series
of
30
women
who
had
45
pregnancies
.
Patients
completed
a
specific
questionnaire
concerning
MC
mediator
release
symptoms
graded
according
to
their
frequency
to
detect
clinical
changes
occurring
during
pregestation
and
pregnancy
as
well
as
postpartum
.
Information
about
the
medications
received
during
pregnancy
and
labor
and
about
newborn
medical
complications
was
also
recorded
.
Worsening
of
MC
-related
symptoms
during
pregnancy
was
observed
in
10
cases
(
22
%
)
;
additionally
,
1
woman
developed
skin
lesions
as
a
manifestation
of
indolent
systemic
mastocytosis
(
ISM
)
within
the
third
trimester
of
pregnancy
.
Conversely
,
15
cases
(
33
%
)
experienced
clinical
improvement
during
pregnancy
,
with
a
complete
resolution
of
pregestational
symptoms
in
7
cases
.
MC
mediator
release
symptoms
intrapartum
were
observed
in
5
cases
(
11
%
)
without
any
fatal
outcome
.
Newborn
medical
complications
(
e
.
g
.
prematurity
,
low
birth
weight
,
and
respiratory
distress
)
were
detected
in
7
infants
(
16
%
)
who
were
all
successfully
managed
with
conservative
measures
.
One
infant
developed
cutaneous
mastocytosis
several
years
after
birth
.
Mastocytosis
has
a
heterogeneous
clinical
behavior
during
pregnancy
:
the
profile
of
MC
-related
symptoms
remained
unchanged
in
half
of
the
cases
,
while
in
the
other
half
pregnant
women
experienced
either
an
improvement
or
an
exacerbation
of
the
symptoms
,
with
the
manifestation
of
ISM
during
pregnancy
in
1
case
.
To
prevent
potential
life-threatening
MC
-related
symptoms
,
adequate
prophylactic
antimediator
therapy
intrapartum
should
be
systematically
administered
.
The
absence
of
both
maternal
and
infant
severe
complications
suggests
that
patients
with
nonaggressive
categories
of
mastocytosis
should
not
be
advised
against
pregnancy
.
Diseases
Validation
Diseases presenting
"specific questionnaire"
symptom
cutaneous mastocytosis
dystrophic epidermolysis bullosa
fabry disease
lamellar ichthyosis
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