1. INTRODUCTION
Mastocytosis is a rare disease caused by the abnormal proliferation of mast cells. The prevalence of mastocytosis is estimated to be 13 per 100000 in all age categories [1,2]. Systemic mastocytosis is a genetic disorder and is most commonly caused by a point mutation in the gene for tyrosine kinase receptor Kit D816 V. The symptoms of mastocytosis are quite diverse as mast cells accumulate in various organs, especially the bone marrow, skin, liver, spleen, and lymph nodes. Histamine, heparin, and tryptase are the most recognized preformed mediators that are released from mast cells. Newly formed mediators at the time of the reaction include prostaglandins, leukotrienes, chemokines and cytokines [3]. Mast cell mediator release can lead to general symptoms such as abdominal pain, urticaria, pruritus, syncope, and vascular collapse, including death in severe cases. Common triggers are insect stings and drugs, including nonsteroidal anti-inflammatory drugs (NSAIDs), anesthetics, antibiotics, food, and mechanical stimulation [2]. Stress and pain during labor and delivery can activate the disease [3, 4]. Here, we report the case of a pregnant woman with systemic mastocytosis who received epidural analgesia to avoid labor pain.