成人朗格汉斯细胞组织细胞增生症的诊疗

doi:10.3969/j.issn.1004-583X.2021.10.008

临床荟萃 ›› 2021, Vol. 36 ›› Issue (10): 905-909.doi: 10.3969/j.issn.1004-583X.2021.10.008

• 专题:血液病诊疗关键技术转化与推广 • 上一篇下一篇

成人朗格汉斯细胞组织细胞增生症的诊疗

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:2020-10-08 出版日期:2021-10-20 发布日期:2021-11-10
通讯作者: 曹欣欣 E-mail:caoxinxin@126.com
作者简介:曹欣欣,北京协和医院血液内科副主任医师、副教授、硕士生导师,2005年毕业于中国协和医科大学,医学博士。担任中华医学会血液学会青委委员、北京医学会血液学会青委委员、北京医师协会血液内科专科医师分会理事、中国医师协会组织细胞疾病委员会委员、中国抗癌协会血液肿瘤专业委员会青年委员。主要研究方向:浆细胞与组织细胞肿瘤。
基金资助:
北京市自然科学基金——基于单细胞测序技术对华氏巨球蛋白血症细胞异质性以及发病机制的研究(7202160);中国医学科学院基本科研业务费健康长寿项目——建立基于综合老年学评估和生物学危险分层的老年急性髓系白血病个体化治疗新策略(2019-RC-HL-001)

Diagnosis and treatment of adult Langerhans cell histiocytosis

Cao Xinxin()

Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China

Received:2020-10-08 Online:2021-10-20 Published:2021-11-10
Contact: Cao Xinxin E-mail:caoxinxin@126.com

摘要/Abstract

摘要：

朗格汉斯细胞组织细胞增生症(LCH)是一种以MAPK信号通路激活为主要特征的克隆性血液系统肿瘤,属于炎性髓系肿瘤。其临床表现多样,最常见的临床受累脏器为骨骼、肺、垂体等。其主要依赖于病理活检确诊,病理免疫组化CD207和电镜检查可见伯贝克颗粒是诊断的金标准。根据临床受累脏器范围,LCH分为单系统单病灶、单系统多病灶、多系统受累。治疗主要根据临床分型,对于单系统单病灶患者以局部治疗为主,对于单系统多病灶或多系统LCH以全身治疗为主。

关键词: 组织细胞增多症,朗格汉斯细胞, MAPK信号通路, 诊断, 治疗

Abstract:

Langerhans cell histiocytosis(LCH) is an inflammatory myeloid malignancies with MAPK pathway activation mutations detectable in nearly all patients and a pronounced inflammatory state. The clinical manifestations of LCH are diversity, the most common involved organs included Bones, lungs, pituitary, ect. The primary diagnosis of LCH depends on histopathologic review of lesional tissue biopsy; the gold standard for diagnosis were expression of Cd207 (Langerlin) in immunohistochemistry and birbeck granules(BGs) by electron microscopy. LCH can be devided into localized single-system disease(SS-s), multiple sites within one single system (SS-m), and multisystem(MS) disease according to the scope of clinically involved organs. In terms of clinical classification, Local therapies may be sufficient for SS-s patients, systemic therapies might be better for SS-m or MS patients.

Key words: histiocytosis,langerhans-cell, MAPK pathway, diagnosis, treatment

中图分类号:

R551.1

曹欣欣. 成人朗格汉斯细胞组织细胞增生症的诊疗[J]. 临床荟萃, 2021, 36(10): 905-909.

Cao Xinxin. Diagnosis and treatment of adult Langerhans cell histiocytosis[J]. Clinical Focus, 2021, 36(10): 905-909.

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链接本文: http://www.lchc.cn/CN/10.3969/j.issn.1004-583X.2021.10.008

http://www.lchc.cn/CN/Y2021/V36/I10/905

图/表 4

图1 PET/CT:左下颌骨、左股骨颈高代谢

图2 头颅CT及垂体MRI:右顶骨骨质破坏伴软组织肿物

图3 垂体增强MRI:鞍上占位,T2强化

图4 胸部HRCT:双肺间质纹理增多,弥漫薄壁囊腔

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成人朗格汉斯细胞组织细胞增生症的诊疗

Diagnosis and treatment of adult Langerhans cell histiocytosis

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