3. DISCUSSION
We present a case of pregnancy complicated by the need for the
management of systemic mastocytosis with several prior anaphylaxis
episodes. We applied epidural analgesia to prevent exacerbation of the
disease and anaphylaxis during labor and delivery. Multiple stimuli can
provoke mast cell degranulation. The triggers include physical, chemical
and biologic agents [3,5]. Stress is also a well-known trigger for
mediator release in systemic mastocytosis [3,4]. Pregnancy
complications can be associated with increased levels of maternal
stress, and labor is clearly a time of increased maternal pain and
stress [3]. Based on the hypothesis that good pain management may
contribute to reducing stress and reducing the risk of exacerbation of
systemic mastocytosis, there are several prior reports of epidural
anesthesia for pain relief during labor in pregnant patients with
systemic mastocytosis. [6,7]. We planned early epidural placement to
avoid stress and labor pain, which can trigger systemic mastocytosis.
However, anaphylactoid reactions may also be triggered by drugs
administered during delivery, especially anesthesia. Testing for drug
hypersensitivity is generally recommended only in patients with a
history of drug allergy [8]. This patient had a history of drug
allergy. However, in this case, labor analgesics and general anesthetics
were not tested during pregnancy while planning the labor analgesia
because the allergy/immunology physician did not recommend it as the
testing could trigger mast cell degranulation. Therefore, we avoided the
use of anesthetics such as morphine, NSADs, meperdine, and ester local
anesthetics, which could trigger mast cell degranulation during labor
analgesia [9].
This patient had an NRS score of 10 at the time she was admitted to the
hospital and complained of very strong pain. Although it should have
been introduced by the straight epidural method assuming the possibility
of fetal distress and a possible emergency cesarean section, the CSE
method was selected by the anesthesiologist in charge because CSE was
the most commonly used method. As our facility uses PIEB as a standard,
the level of anesthesia and her vitality were checked every hour to
avoid unexpected hypotension or high epidural levels.
Mastocytosis is subdivided into two categories as proposed by the WHO:
cutaneous and systemic mastocytosis [10,11]. Cutaneous mastocytosis
is the most common form of mastocytosis, which usually occurs in
childhood and appears as hyperplasia of the mast cells of the skin
[12]. Systemic mastocytosis is a very rare disease in which
myeloproliferative tumors derived from mast cells form multifocal
lesions and often infiltrate the skin, lymph nodes, digestive organs,
liver, and spleen. The diagnosis of systemic mastocytosis is based on
the presence of one major criterion and one minor criterion or three
minor criteria (Table 1). Symptoms of systemic mastocytosis include
abdominal pain, diarrhea, nausea, vomiting, skin pruritus and flushing,
anaphylactoid reactions, anemia, and pathological fractures. Our patient
was first diagnosed with urticaria pigmentosa, but later, she was
diagnosed with systemic mastocytosis by observing the proliferation of
mast cells in skin and bone marrow biopsies.
Approximately 22% to 33% of pregnant women with systemic mastocytosis
experience worsening of systemic symptoms due to increased physical and
psychological stress during pregnancy [6,9,13]. Theoretically,
hormonal changes associated with pregnancy and puerperium may promote
anaphylactic reactions. There are reports of anaphylactic episodes
during pregnancy [14]. Exacerbations of systemic mastocytosis during
pregnancy can cause life-threatening maternal and fetal complications.
There have been several reports of preterm labor and deliveries
[7,9]. There is also a case report of a fetal death due to an acute
exacerbation of systemic mastocytosis [3]. This fetal demise that
occurred at 31 weeks of gestation was caused by an exacerbation of
systemic mastocytosis, which resulted in severe hypotension and a
critical decrease in uterine blood flow.
In our case, there were no significant changes during pregnancy. The
anesthesiologist should prepare a plan for analgesia and anesthesia for
delivery to minimize the risk. Good pain control helps reduce overall
anxiety and reduces the risk of exacerbated mastocytosis during labor.
Our patient presented with minor skin symptoms during labor pain, but it
improved with antihistamines and immediate pain relief from epidural
analgesia without any serious complications.