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成人朗格汉斯细胞组织细胞增多症伴多系统骨骼、皮肤、肺和肝脏受累:一例报告。

Adult Langerhans cell histiocytosis with multi‑system bone, skin, lung and liver involvement: A case report.

作者信息

Lei Qiucheng, Hong Xitao, Yuan Jianxiang, Zheng Huazhen, Deng Feiwen

机构信息

Organ Transplant Center, The First People's Hospital of Foshan, Foshan, Guangdong 528000, P.R. China.

School of Medicine, Southeast University, Nanjing, Jiangsu 210009, P.R. China.

出版信息

Biomed Rep. 2024 Sep 2;21(5):162. doi: 10.3892/br.2024.1850. eCollection 2024 Nov.

Abstract

Langerhans cell histiocytosis (LCH) is a rare neoplastic disorder marked by the uncontrolled proliferation and accumulation of immature myeloid dendritic cells, which originate from the bone marrow. Although LCH can involve various organs, including bone, lymph nodes and skin, multi-system bone, liver and lung involvement with LCH is rare in adults. A case of a 49-year-old man diagnosed with multi-system, aggressive LCH involving bone, skin, lung and liver is presented in the present study. The initial radio-clinic presentation of the patient was initially suggestive of a bone tumor. The current case report aims to draw attention to this rare disease and discuss the diagnostic approach and therapeutic management, which should be noted to help physicians more rapidly identify, diagnose and treat comparable cases.

摘要

朗格汉斯细胞组织细胞增多症(LCH)是一种罕见的肿瘤性疾病,其特征为起源于骨髓的未成熟髓样树突状细胞不受控制地增殖和积聚。尽管LCH可累及多个器官,包括骨骼、淋巴结和皮肤,但成人中多系统骨骼、肝脏和肺部受累的LCH较为罕见。本研究报告了一例49岁男性被诊断为多系统侵袭性LCH,累及骨骼、皮肤、肺部和肝脏的病例。该患者最初的放射学和临床症状最初提示为骨肿瘤。本病例报告旨在引起对这种罕见疾病的关注,并讨论诊断方法和治疗管理,值得注意的是,这有助于医生更快地识别、诊断和治疗类似病例。

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Langerhans-Cell Histiocytosis.朗格汉斯细胞组织细胞增多症
N Engl J Med. 2018 Aug 30;379(9):856-868. doi: 10.1056/NEJMra1607548.

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