Escribano Luis, Akin Cem, Castells Mariana, Schwartz Lawrence B
Unidad de Mastocitosis, Laboratorio K.Frank Austen, Hospital Ramón y Cajal, Red Española de Mastocitosis, Madrid, Spain.
Inflamm Allergy Drug Targets. 2006 Jan;5(1):61-77. doi: 10.2174/187152806775269303.
Patients with mastocytosis have symptoms related to the tissue response to the release of mediators from mast cells (MC), local mast cell burden or associated non-mast cell hematological disorders. MC contain an array of biologically active mediators in their granules, which are preformed and stored. MC are also able to produce newly generated membrane-derived lipid mediators and are a source of multifunctional cytokines. Mediator-related symptoms can include pruritus, flushing, syncope, gastric distress, nausea and vomiting, diarrhea, bone pain and neuropsychiatric disturbances; these symptoms are variably controlled by adequate medications. Management of patients within all categories of mastocytosis includes: a) a careful counseling of patients (parents in pediatric cases) and care providers, b) avoidance of factors triggering acute mediator release, c) treatment of acute and chronic MC-mediator symptoms and, if indicated, d) an attempt for cytoreduction and treatment of organ infiltration by mast cells.
肥大细胞增多症患者的症状与组织对肥大细胞（MC）释放介质的反应、局部肥大细胞负荷或相关的非肥大细胞血液系统疾病有关。MC在其颗粒中含有一系列生物活性介质，这些介质是预先形成并储存的。MC还能够产生新生成的膜衍生脂质介质，并且是多功能细胞因子的来源。与介质相关的症状可包括瘙痒、潮红、晕厥、胃部不适、恶心和呕吐、腹泻、骨痛及神经精神障碍；这些症状可通过适当的药物得到不同程度的控制。所有类型肥大细胞增多症患者的管理包括：a）对患者（儿科病例中的家长）和护理人员进行仔细的咨询，b）避免触发急性介质释放的因素，c）治疗急性和慢性MC介质症状，以及在有指征时d）尝试进行细胞减少并治疗肥大细胞的器官浸润。
