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对包括肥大细胞增多症在内的克隆性肥大细胞疾病的新见解。

New Insights into Clonal Mast Cell Disorders Including Mastocytosis.

作者信息

Volertas Sofija, Schuler Charles F, Akin Cem

机构信息

Division of Allergy and Clinical Immunology, Department of Internal Medicine, University of Michigan, 24 Frank Lloyd Wright Drive, PO Box 442, Suite H-2100, Ann Arbor, MI 48106-0442, USA.

Division of Allergy and Clinical Immunology, Department of Internal Medicine, University of Michigan, 24 Frank Lloyd Wright Drive, PO Box 442, Suite H-2100, Ann Arbor, MI 48106-0442, USA.

出版信息

Immunol Allergy Clin North Am. 2018 Aug;38(3):341-350. doi: 10.1016/j.iac.2018.04.014. Epub 2018 Jun 9.

Abstract

Mastocytosis is a heterogeneous group of neoplasms that involve the clonal expansion of mast cells into one or more organ systems, which typically involves the skin and hematopoietic systems. Systemic mastocytosis consists of a multifocal infiltration of mast cells into various noncutaneous tissue sites, especially the bone marrow. Diagnosis requires tissue confirmation, and algorithms have been developed to assist clinicians in this process. The current classification system focuses on delineating prognostic categories. Therapeutic approaches include symptomatic management, prevention of complications, and, in advanced disease, cytoreductive therapy.

摘要

肥大细胞增多症是一组异质性肿瘤,涉及肥大细胞克隆性扩增至一个或多个器官系统,通常累及皮肤和造血系统。系统性肥大细胞增多症表现为肥大细胞多灶性浸润至各种非皮肤组织部位,尤其是骨髓。诊断需要组织学证实,并且已经开发出算法来协助临床医生进行这一过程。当前的分类系统侧重于划分预后类别。治疗方法包括对症处理、预防并发症,以及在晚期疾病中进行减瘤治疗。

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