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肥大细胞活化紊乱谱

Spectrum of mast cell activation disorders.

作者信息

Petra Anastasia I, Panagiotidou Smaro, Stewart Julia M, Conti Pio, Theoharides Theoharis C

机构信息

Department of Molecular Physiology and Pharmacology, Molecular Immunopharmacology and Drug Discovery Laboratory, Tufts University School of Medicine and Tufts Medical Center, 136 Harrison Avenue, Boston, MA, USA.

出版信息

Expert Rev Clin Immunol. 2014 Jun;10(6):729-39. doi: 10.1586/1744666X.2014.906302. Epub 2014 May 1.

DOI:10.1586/1744666X.2014.906302
PMID:24784142
Abstract

Mast cell (MC) activation disorders present with multiple symptoms including flushing, pruritus, hypotension, gastrointestinal complaints, irritability, headaches, concentration/memory loss and neuropsychiatric issues. These disorders are classified as: cutaneous and systemic mastocytosis with a c-kit mutation and clonal MC activation disorder, allergies, urticarias and inflammatory disorders and mast cell activation syndrome (MCAS), idiopathic urticaria and angioedema. MCs are activated by IgE, but also by cytokines, environmental, food, infectious, drug and stress triggers, leading to secretion of multiple mediators. The symptom profile and comorbidities associated with these disorders, such as chronic fatigue syndrome and fibromyalgia, are confusing. We propose the use of the term 'spectrum' and highlight the main symptoms, useful diagnostic tests and treatment approaches.

摘要

肥大细胞(MC)活化障碍表现为多种症状,包括潮红、瘙痒、低血压、胃肠道不适、易怒、头痛、注意力/记忆力减退以及神经精神问题。这些障碍分类如下:伴有c-kit突变的皮肤性和系统性肥大细胞增多症以及克隆性MC活化障碍、过敏、荨麻疹和炎症性疾病、肥大细胞活化综合征(MCAS)、特发性荨麻疹和血管性水肿。MC由IgE激活,但也可由细胞因子、环境、食物、感染、药物和应激触发因素激活,从而导致多种介质的分泌。与这些障碍相关的症状谱和合并症,如慢性疲劳综合征和纤维肌痛,令人困惑。我们建议使用“谱系”一词,并突出主要症状、有用的诊断测试和治疗方法。

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1
Spectrum of mast cell activation disorders.肥大细胞活化紊乱谱
Expert Rev Clin Immunol. 2014 Jun;10(6):729-39. doi: 10.1586/1744666X.2014.906302. Epub 2014 May 1.
2
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Clinical, biological, and molecular characteristics of clonal mast cell disorders presenting with systemic mast cell activation symptoms.伴有全身肥大细胞活化症状的克隆性肥大细胞疾病的临床、生物学和分子特征。
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Pathogenic and diagnostic relevance of KIT in primary mast cell activation disorders.原发性肥大细胞激活障碍中 KIT 的发病机制和诊断相关性。
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Clinical, immunophenotypic, and molecular characteristics of well-differentiated systemic mastocytosis.系统肥大细胞分化良好的临床、免疫表型和分子特征。
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J Allergy Clin Immunol. 2018 Jan;141(1):180-188.e3. doi: 10.1016/j.jaci.2017.05.036. Epub 2017 Jun 16.
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Mastocytosis: state of the art.肥大细胞增多症:最新进展
Pathobiology. 2007;74(2):121-32. doi: 10.1159/000101711.
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Immunoreactivity for CD25 in gastrointestinal mucosal mast cells is specific for systemic mastocytosis.胃肠道黏膜肥大细胞中CD25的免疫反应性是系统性肥大细胞增多症所特有的。
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Transglutaminase 2 expressed in mast cells recruited into skin or bone marrow induces the development of pediatric mastocytosis.在募集到皮肤或骨髓中的肥大细胞中表达的转谷氨酰胺酶2会诱导小儿肥大细胞增多症的发展。
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Systemic mastocytosis.系统性肥大细胞增生症。
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