| 张 敏,翟志芳,宋志强,罗 雪,余 佳.肥大细胞增生症研究进展[J].,2017,17(20):3987-3991 |
| 肥大细胞增生症研究进展 |
| Research Progress of Mastocytosis |
| 投稿时间：2017-02-13 修订日期：2017-03-09 |
| DOI：10.13241/j.cnki.pmb.2017.20.044 |
| 中文关键词: 肥大细胞 肥大细胞增生症 系统性肥大细胞增生症 治疗 |
| 英文关键词: Mast cell Mastocytosis Systemic mastocytosis Treatment |
| 基金项目:国家自然科学基金项目（81302413） |
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| 摘要点击次数: 237 |
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| 中文摘要: |
| 摘要：肥大细胞增生症（mastocytosis）是由多种临床表现组成的一组疾病谱，其特征表现以肥大细胞在一个或多个器官异常浸润 和积聚为主，常累及皮肤、消化道、骨髓、肝脾以及淋巴结，临床表现为皮肤瘙痒、潮红、恶心、呕吐、腹泻、腹痛等改变。针对存在以下情况需考虑本病：不明原因的溃疡或吸收不良；影像学检查发现骨骼扫描异常、肝脾肿大、淋巴结肿大；外周血异常；速发型过敏反应；或原因不明的低血压；一旦确定为肥大细胞增生症引起，应对疾病活动性、亚型和预后进行评估，并进行适当治疗；肥大细胞增多症根据临床表现、病理改变和预后分为7种不同类型，皮肤性及惰性系统性肥大细胞增多症预后较好，肥大细胞肉瘤预后最差。本文对肥大细胞增生症发病机制、临床特征、诊断、治疗及预后等方面的研究现状进行综述。 |
| 英文摘要: |
| ABSTRACT: Mastocytosis is a group of spectrum which consists of a variety of clinical manifestations, with the mainly character of mast cells abnormal infiltration and accumulation in one or more organs. Skin, digestive tract, bone marrow, liver, spleen and lymph node are the common organs involved. Clinical manifestations includes the change of pruritus, facial flush, nausea, vomiting, abdominal pain, diarrhea, etc. Since there are following situations, these diseases should be considered, such as, unidentified reasons' ulcer or malabsorption; imaging examination showed abnormal bone scan, hepatosplenomegaly, enlarged lymph glands; peripheral blood abnomaly; anaphylaxis; or unexplained hypotension; once it is determined caused by mastocytosis, assessment can be performed at disease activity, subtypes and prognosis, and with proper treatment; Mastocytosis is divided into seven different kinds, according to clinical manifestations, pathological changes and prognosis, skin and inert systemic mastocytosis has better prognosis, prognosis of mastocytoma is worst.This article mainly summarized the current research on pathogenesis, clinical features, diagnosis, treatment and prognosis of mastocytosis, etc. |
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