Rosai-Dorfman病的诊治进展

doi:10.16352/j.issn.1001-6325.2022.11.1785

基础医学与临床 ›› 2022, Vol. 42 ›› Issue (11): 1785-1790.doi: 10.16352/j.issn.1001-6325.2022.11.1785

Rosai-Dorfman病的诊治进展

刘婷, 曹欣欣^*

中国医学科学院北京协和医学院北京协和医院血液内科, 北京 100730

收稿日期:2021-08-06 修回日期:2021-12-08 出版日期:2022-11-05 发布日期:2022-11-01
通讯作者: ^* caoxinxin@pumch.cn
基金资助:
北京市自然科学基金(7202160); 中国医学科学院基本科研业务费健康长寿项目(2019-RC-HL-001)

Progress in the diagnosis and treatment of Rosai-Dorfman disease

LIU Ting, CAO Xin-xin^*

Department of Hematology, Peking Union Medical College Hospital, CAMS & PUMC, Beijing 100730, China

Received:2021-08-06 Revised:2021-12-08 Online:2022-11-05 Published:2022-11-01
Contact: ^* caoxinxin@pumch.cn

摘要/Abstract

摘要： 罗道病(RDD)是一种罕见的非朗格汉斯细胞组织细胞增生症,其病因及发病机制尚未完全阐明,临床表现具有高度异质性, 极易误诊、漏诊,目前尚无统一的治疗方法。本文通过文献回顾,就近年来有关RDD的发病机制、诊断和治疗进展进行综述,并总结了RDD的治疗原则。RDD的治疗需个体化,对于单纯淋巴结型或仅皮肤受累的无症状RDD患者,可先予观察,密切随诊;对于结外孤立性病灶或造成局部器官压迫的RDD患者,建议手术切除病灶;对于全身性及复发难治性RDD患者,可考虑全身药物治疗。

关键词: Rosai-Dorfman病, 临床表现, 治疗

Abstract: Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytosis. Its etiology and pathogenesis are yet incompletely elucidated. The clinical manifestations of RDD are highly heterogeneous. It is easy to be misdiagnosed or missed diagnosis. There is no standard treatment at present. Therefore, the progression in pathogenesis, diagnosis and treatment are reviewed. The treatment of RDD is best tailored to the individual clinical circumstances. Observation is suitable for patients with uncomplicated lymphadenopathy or asymptomatic cutaneous RDD. For RDD patients with extra nodal unifocal disease or organ compression, surgery is recommended. Systemic therapy can be considered for patients with systemic, recurrent or refractory RDD.

Key words: Rosai-Dorfman disease, clinical manifestations, treatment

中图分类号:

R559

刘婷, 曹欣欣. Rosai-Dorfman病的诊治进展[J]. 基础医学与临床, 2022, 42(11): 1785-1790.

LIU Ting, CAO Xin-xin. Progress in the diagnosis and treatment of Rosai-Dorfman disease[J]. Basic & Clinical Medicine, 2022, 42(11): 1785-1790.

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Rosai-Dorfman病的诊治进展

Progress in the diagnosis and treatment of Rosai-Dorfman disease

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