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[系统性肥大细胞增多症——一种内科疾病的定义]

[Systemic mastocytosis--definition of an internal disease].

作者信息

Homann Jürgen, Kolck Ulrich W, Ehnes Andreas, Frieling Thomas, Raithel Martin, Molderings Gerhard J

机构信息

Abteilung für Innere Medizin, Evangelisches Waldkrankenhaus, Bonn, Germany.

出版信息

Med Klin (Munich). 2010 Aug;105(8):544-53. doi: 10.1007/s00063-010-1093-8. Epub 2010 Sep 8.

Abstract

Systemic mastocytosis comprises disorders characterized by an accumulation of genetically altered mast cells in all organs and tissues due to an increased proliferation rate and reduced apoptosis of those pathologic mast cells. Release of their mediators can effectively influence organ function and can lead to systemic effects without inducing traces in routinely used laboratory parameters or imaging methods. In most cases, little invasive investigations allow diagnosing the disease and, hence, an appropriate therapy consisting of a basic medication with antihistamine and mast cell membrane-stabilizing compounds that should be supplemented, if required, by a medication adapted to individual symptoms, can be initiated. Because of the probably high prevalence of the disorder, systemic mastocytosis should be considered as a differential diagnosis in particular in the case of chronic gastrointestinal complaints such as abdominal pain/discomfort possibly associated with diarrhea, at an early stage.

摘要

系统性肥大细胞增多症包括一些疾病,其特征是由于病理肥大细胞增殖率增加和凋亡减少,导致基因改变的肥大细胞在所有器官和组织中积聚。这些肥大细胞释放的介质可有效影响器官功能,并可导致全身效应,而在常规实验室参数或成像方法中不留下痕迹。在大多数情况下,通过微创检查即可诊断该病,因此,可以开始进行适当的治疗,包括使用抗组胺药和肥大细胞膜稳定化合物的基础药物,如有需要,可辅以根据个体症状调整的药物。由于该疾病的患病率可能很高,特别是在出现慢性胃肠道症状(如可能与腹泻相关的腹痛/不适)的早期阶段,应将系统性肥大细胞增多症视为鉴别诊断的考虑对象。

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