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肥大细胞增多症的标准与标准化:关于诊断、治疗建议及反应标准的共识声明

Standards and standardization in mastocytosis: consensus statements on diagnostics, treatment recommendations and response criteria.

作者信息

Valent P, Akin C, Escribano L, Födinger M, Hartmann K, Brockow K, Castells M, Sperr W R, Kluin-Nelemans H C, Hamdy N A T, Lortholary O, Robyn J, van Doormaal J, Sotlar K, Hauswirth A W, Arock M, Hermine O, Hellmann A, Triggiani M, Niedoszytko M, Schwartz L B, Orfao A, Horny H-P, Metcalfe D D

机构信息

Department of Internal Medicine I, Division of Haematology and Haemostaseology, Medical University of Vienna, Vienna, Austria.

出版信息

Eur J Clin Invest. 2007 Jun;37(6):435-53. doi: 10.1111/j.1365-2362.2007.01807.x.

Abstract

Although a classification for mastocytosis and diagnostic criteria are available, there remains a need to define standards for the application of diagnostic tests, clinical evaluations, and treatment responses. To address these demands, leading experts discussed current issues and standards in mastocytosis in a Working Conference. The present article provides the resulting outcome with consensus statements, which focus on the appropriate application of clinical and laboratory tests, patient selection for interventional therapy, and the selection of appropriate drugs. In addition, treatment response criteria for the various clinical conditions, disease-specific symptoms, and specific pathologies are provided. Resulting recommendations and algorithms should greatly facilitate the management of patients with mastocytosis in clinical practice, selection of patients for therapies, and the conduct of clinical trials.

摘要

虽然已有肥大细胞增多症的分类和诊断标准,但仍需要为诊断测试的应用、临床评估和治疗反应确定标准。为满足这些需求,顶尖专家在一次工作会议上讨论了肥大细胞增多症的当前问题和标准。本文给出了会议结果及共识声明,重点关注临床和实验室检查的合理应用、介入治疗的患者选择以及合适药物的选择。此外,还提供了针对各种临床情况、疾病特异性症状和特定病理的治疗反应标准。由此得出的建议和算法应能极大地促进临床实践中肥大细胞增多症患者的管理、治疗患者的选择以及临床试验的开展。

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