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.