J Investig Allergol Clin Immunol. 2014;24(5):288-97; quiz 3 p preceding 297.
Mastocytosis is a clonal disorder characterized by proliferation and accumulation of mast cells in various tissues, mainly skin and bone marrow. It can cause a wide variety of clinical manifestations-other than urticaria pigmentosa-that can lead to inappropriate release of mediators by mast cells. The most severe manifestation is anaphylaxis. The triggers of anaphylaxis in adults with mastocytosis are numerous, but Hymenoptera stings seem to be the most frequent, followed by foods and drugs. Therefore, to prevent severe reactions, it is very important to recognize and avoid potential triggers; in addition, venom-allergic patients must receive lifelong immunotherapy, which has proven very effective. Given that published data on drug anaphylaxis in patients with mast cell disorders are scarce, it is not currently possible to provide clear recommendations. The risk of systemic reactions during general anesthesia can be reduced by assessing risk on an individual basis (previous reaction to a drug or reaction during surgery) and by avoiding specific trigger factors (patient temperature changes, infusion of cold solution, tissue trauma, friction, and other mechanical factors).
肥大细胞增多症是一种以肥大细胞在各种组织（主要是皮肤和骨髓）中增殖和积聚为特征的克隆性疾病。它可引起多种除色素性荨麻疹以外的临床表现，导致肥大细胞释放不适当的介质。最严重的表现是过敏反应。成人肥大细胞增多症患者发生过敏反应的诱因很多，但蜂类蜇伤似乎最为常见，其次是食物和药物。因此，为了防止严重反应，识别和避免潜在的诱因非常重要；此外，毒液过敏患者必须接受终身免疫治疗，这已被证明非常有效。鉴于关于肥大细胞疾病患者药物过敏反应的已发表数据很少，目前还无法提供明确的建议。通过评估个体的风险（对药物的先前反应或手术期间的反应）和避免特定的触发因素（患者体温变化、输注冷溶液、组织创伤、摩擦和其他机械因素），可以降低全身麻醉期间发生系统性反应的风险。
