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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.

DOI:10.3892/br.2024.1850
PMID:39268400
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11391176/
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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本文引用的文献

1
Langerhans cell histiocytosis: current advances in molecular pathogenesis.朗格汉斯细胞组织细胞增生症:分子发病机制的最新进展。
Front Immunol. 2023 Oct 26;14:1275085. doi: 10.3389/fimmu.2023.1275085. eCollection 2023.
2
Clinical and prognostic features of Langerhans cell histiocytosis in adults.成人朗格汉斯细胞组织细胞增生症的临床和预后特征。
Cancer Sci. 2023 Sep;114(9):3687-3697. doi: 10.1111/cas.15879. Epub 2023 Jun 26.
3
Treatment outcomes and prognostic factors of patients with adult Langerhans cell histiocytosis.成人朗格汉斯细胞组织细胞增生症患者的治疗结果和预后因素。
Am J Hematol. 2022 Feb 1;97(2):203-208. doi: 10.1002/ajh.26412. Epub 2021 Nov 23.
4
Treatment Outcomes of Langerhans Cell Histiocytosis: A Retrospective Study.朗格汉斯细胞组织细胞增多症的治疗结果:一项回顾性研究
Medicina (Kaunas). 2021 Apr 7;57(4):356. doi: 10.3390/medicina57040356.
5
Unusual sites of bone involvement in Langerhans cell histiocytosis: a systematic review of the literature.朗格汉斯细胞组织细胞增生症中骨骼受累的不常见部位:文献系统回顾。
Orphanet J Rare Dis. 2021 Jan 2;16(1):1. doi: 10.1186/s13023-020-01625-z.
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Langerhans Cell Histiocytosis with Uncommon Liver Involvement: A Case Report.朗格汉斯细胞组织细胞增生症伴肝内罕见受累:病例报告。
Am J Case Rep. 2020 Nov 15;21:e923505. doi: 10.12659/AJCR.923505.
7
Langerhans cell histiocytosis with BRAF p.N486_P490del or MAP2K1 p.K57_G61del treated by the MEK inhibitor trametinib.朗格汉斯细胞组织细胞增生症伴 BRAF p.N486_P490del 或 MAP2K1 p.K57_G61del,用 MEK 抑制剂曲美替尼治疗。
Pediatr Blood Cancer. 2020 Dec;67(12):e28712. doi: 10.1002/pbc.28712. Epub 2020 Sep 29.
8
High levels of plasma interleukin-17A are associated with severe neurological sequelae in Langerhans cell histiocytosis.血浆白细胞介素-17A 水平升高与朗格汉斯细胞组织细胞增生症的严重神经后遗症有关。
Cytokine. 2020 Feb;126:154877. doi: 10.1016/j.cyto.2019.154877. Epub 2019 Oct 16.
9
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.
10
Long-term disease control of Langerhans cell histiocytosis using combined BRAF and MEK inhibition.采用联合 BRAF 和 MEK 抑制治疗朗格汉斯细胞组织细胞增生症的长期疾病控制。
Blood Adv. 2018 Aug 28;2(16):2156-2158. doi: 10.1182/bloodadvances.2018021782.