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以肝肿瘤破裂为表现的成人多系统朗格汉斯细胞组织细胞增生症的自发消退

Spontaneous Regression of Adult Multi-system Langerhans Cell Histiocytosis Presenting as Liver Tumor Rupture.

作者信息

Oshima Seigi, Inano Shojiro, Honjo Gen, Tabata Sumie, Fujimoto Masakazu, Haga Hironori, Kitano Toshiyuki

机构信息

Department of Hematology, Medical Research Institute KITANO HOSPITAL, PIIF Tazuke-kofukai, Japan.

Department of Hematology, Kyoto University, Japan.

出版信息

Intern Med. 2025 Apr 15;64(8):1217-1222. doi: 10.2169/internalmedicine.4060-24. Epub 2024 Sep 4.

DOI:10.2169/internalmedicine.4060-24
PMID:39231671
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12097838/
Abstract

Adult multisystem Langerhans cell histiocytosis (MS-LCH) is rare and has a poor prognosis. A 67-year-old man with MS-LCH presented with a hepatic tumor rupture and multiple masses in the lungs, liver, and pancreas. Despite the initial aggressive disease course and involvement of organs at risk, the patient experienced spontaneous regression and lesion disappearance following smoking cessation without chemotherapy. A literature review revealed a distinct subset of MS-LCH that can be managed by smoking cessation and careful observation through follow-up imaging. This suggests that careful observation through follow-up imaging may be a reasonable alternative to chemotherapy in select adult cases of MS-LCH.

摘要

成人多系统朗格汉斯细胞组织细胞增多症(MS-LCH)较为罕见,预后较差。一名67岁的MS-LCH男性患者出现肝肿瘤破裂,肺部、肝脏和胰腺有多处肿块。尽管疾病初期进展迅速且累及危险器官,但患者戒烟后未接受化疗,肿瘤却自行消退且病灶消失。文献综述显示,MS-LCH中有一个独特的亚组,可通过戒烟和后续影像学检查进行密切观察来处理。这表明,在部分成人MS-LCH病例中,通过后续影像学检查进行密切观察可能是化疗的合理替代方案。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9dab/12097838/57d1b22c5c5a/1349-7235-64-1217-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9dab/12097838/862a9fee3711/1349-7235-64-1217-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9dab/12097838/5a1cf9c5e43e/1349-7235-64-1217-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9dab/12097838/1b86a4fe89cc/1349-7235-64-1217-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9dab/12097838/2035f1c39071/1349-7235-64-1217-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9dab/12097838/57d1b22c5c5a/1349-7235-64-1217-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9dab/12097838/862a9fee3711/1349-7235-64-1217-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9dab/12097838/5a1cf9c5e43e/1349-7235-64-1217-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9dab/12097838/1b86a4fe89cc/1349-7235-64-1217-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9dab/12097838/2035f1c39071/1349-7235-64-1217-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9dab/12097838/57d1b22c5c5a/1349-7235-64-1217-g005.jpg

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本文引用的文献

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Blood Adv. 2023 Nov 14;7(21):6568-6578. doi: 10.1182/bloodadvances.2023010706.
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Spectrum of second primary malignancies and cause-specific mortality in pediatric and adult langerhans cell histiocytosis.
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Blood. 2022 Apr 28;139(17):2601-2621. doi: 10.1182/blood.2021014343.
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Langerhans-Cell Histiocytosis.朗格汉斯细胞组织细胞增多症
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