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局限性胃朗格汉斯细胞组织细胞增多症患者的临床特征与预后:病例系列研究

Clinical Characteristics and Outcomes in Patients With Localized Gastric Langerhans Cell Histiocytosis: A Case Series.

作者信息

Kim Tae-Se, Ahn Soomin, Min Yang Won, Lee Hyuk, Lee Jun Haeng, Rhee Poong-Lyul, Kim Jae J, Min Byung-Hoon

机构信息

Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

Department of Pathology and Translational Genomics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

出版信息

Korean J Helicobacter Up Gastrointest Res. 2024 Jun;24(2):175-181. doi: 10.7704/kjhugr.2024.0019. Epub 2024 Jun 10.

DOI:10.7704/kjhugr.2024.0019
PMID:40502846
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11967452/
Abstract

OBJECTIVES

Localized gastric Langerhans cell histiocytosis (LCH) characterized by abnormal proliferation of Langerhans cells in the stomach without systemic involvement, is rare; therefore, the clinical characteristics and outcomes of LCH remain unclear. We investigated the clinical characteristics and outcomes in patients diagnosed with localized gastric LCH and have also discussed treatment strategies for this rare disease.

METHODS

The study included seven patients diagnosed with localized gastric LCH at our hospital between September 1997 and December 2023. We retrospectively reviewed medical records and analyzed the clinicopathological characteristics and patient outcomes.

RESULTS

Endoscopically, localized gastric LCH appeared as a small erosion in the distal part of the stomach. Positron emission tomography-computed tomography revealed normal findings in 100.0% (4/4) of patients during pre-treatment workup. Immunohistochemical analysis using S-100 and CD1a showed immunopositive cells in all tested patients. Of the six patients who underwent follow-up, two (33.3%) showed metachronous recurrence at a location distinct from the initial site. However, all patients eventually showed spontaneous regression of the disease, and no gastric LCH-induced mortality was observed during follow-up.

CONCLUSIONS

Careful and regular surveillance may be sufficient for patients with localized gastric LCH without systemic involvement.

摘要

目的

局限性胃朗格汉斯细胞组织细胞增多症(LCH)是一种罕见疾病,其特征为胃内朗格汉斯细胞异常增殖且无全身受累;因此,LCH的临床特征和预后仍不明确。我们调查了诊断为局限性胃LCH患者的临床特征和预后,并讨论了这种罕见疾病的治疗策略。

方法

本研究纳入了1997年9月至2023年12月期间在我院诊断为局限性胃LCH的7例患者。我们回顾性分析了病历,分析了临床病理特征和患者预后。

结果

内镜检查显示,局限性胃LCH表现为胃远端的小糜烂。正电子发射断层扫描-计算机断层扫描显示,100.0%(4/4)的患者在治疗前检查中结果正常。使用S-100和CD1a进行的免疫组织化学分析显示,所有受试患者的免疫阳性细胞均呈阳性。在接受随访的6例患者中,2例(33.3%)在与初始部位不同的位置出现异时性复发。然而,所有患者最终疾病均自发缓解,随访期间未观察到胃LCH导致的死亡。

结论

对于无全身受累的局限性胃LCH患者,仔细且定期的监测可能就足够了。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0756/11967452/107dc4a63605/kjhugr-2024-0019f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0756/11967452/7f0f3ce143f2/kjhugr-2024-0019f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0756/11967452/107dc4a63605/kjhugr-2024-0019f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0756/11967452/7f0f3ce143f2/kjhugr-2024-0019f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0756/11967452/107dc4a63605/kjhugr-2024-0019f2.jpg

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