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15 岁男性患者,以面颈部肿胀为表现的皮下脂膜炎样 T 细胞淋巴瘤。

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) in a 15-Year-old male presenting with orofacial Swelling.

机构信息

Department of Oral and Maxillofacial Surgery, Second Affiliated Hospital of Anhui Medical University, No. 678 Furong Road, Anhui, 230000, Hefei, China.

Department of Hematology, First Affiliated Hospital of Anhui Medical University, No.218 Jixi Road, Anhui, 230000, Hefei, China.

出版信息

BMC Oral Health. 2024 Oct 24;24(1):1280. doi: 10.1186/s12903-024-05045-4.

DOI:10.1186/s12903-024-05045-4
PMID:39448956
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11515410/
Abstract

BACKGROUND

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare form of non-Hodgkin lymphoma that primarily affects subcutaneous tissues. Its occurrence in the orofacial region is exceptionally uncommon, presenting diagnostic challenges due to symptom overlap with more prevalent orofacial condition.

CASE PRESENTATION

This report details the case of a 15-year-old male who presented with persistent left-sided facial swelling, initially misdiagnosed as facial cellulitis. Diagnostic complexity arose from the similarity of symptoms to common orofacial diseases. Comprehensive diagnostic approaches, including liquid-based thin-layer cytometry, immunohistochemistry, and advanced imaging, were pivotal in identifying SPTCL. The recurrence of symptoms following the cessation of dexamethasone treatment indicated hormone dependency. Surgical intervention and subsequent histopathological analysis confirmed SPTCL, with immunohistochemical profiling playing a critical role in the definitive diagnosis. The patient's management involved a multidisciplinary approach, leading to a referral to a hematology specialist and subsequent favorable outcomes.

CONCLUSIONS

This case underscores the diagnostic challenges of orofacial lymphomas such as SPTCL and highlights the necessity for early, accurate pathological diagnosis. It emphasizes the role of advanced diagnostic techniques and the importance of a comprehensive, multidisciplinary approach in the management of such rare cases. This report contributes to the limited but growing body of literature on SPTCL in the orofacial region, providing insights for clinicians in similar future cases.

摘要

背景

皮下脂膜炎样 T 细胞淋巴瘤(SPTCL)是一种罕见的非霍奇金淋巴瘤,主要影响皮下组织。其在口腔颌面部的发生极为罕见,由于与更常见的口腔颌面部疾病的症状重叠,给诊断带来了挑战。

病例介绍

本报告详细介绍了一名 15 岁男性患者的病例,他表现为持续性左侧面部肿胀,最初误诊为面部蜂窝织炎。由于症状与常见的口腔颌面部疾病相似,诊断的复杂性增加了。综合诊断方法,包括液基薄层细胞学、免疫组织化学和先进的影像学检查,对于识别 SPTCL 至关重要。在停止地塞米松治疗后症状再次出现,表明存在激素依赖性。手术干预和随后的组织病理学分析证实了 SPTCL,免疫组织化学分析在明确诊断中发挥了关键作用。患者的管理采用了多学科方法,随后转诊至血液科专家,并取得了良好的结果。

结论

本病例强调了口腔颌面部 SPTCL 等淋巴瘤的诊断挑战,并突出了早期、准确的病理诊断的必要性。它强调了先进诊断技术的作用,以及在管理此类罕见病例中综合、多学科方法的重要性。本报告为口腔颌面部 SPTCL 的有限但不断增长的文献提供了参考,为未来类似病例的临床医生提供了见解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c0ee/11515410/75493f4c2a0a/12903_2024_5045_Fig6_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c0ee/11515410/aaee09b27240/12903_2024_5045_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c0ee/11515410/c6b60787c874/12903_2024_5045_Fig2_HTML.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c0ee/11515410/2e8fc7c9a0ee/12903_2024_5045_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c0ee/11515410/f7aadf0d3289/12903_2024_5045_Fig5_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c0ee/11515410/75493f4c2a0a/12903_2024_5045_Fig6_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c0ee/11515410/aaee09b27240/12903_2024_5045_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c0ee/11515410/c6b60787c874/12903_2024_5045_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c0ee/11515410/03a8fbba03d6/12903_2024_5045_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c0ee/11515410/2e8fc7c9a0ee/12903_2024_5045_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c0ee/11515410/f7aadf0d3289/12903_2024_5045_Fig5_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c0ee/11515410/75493f4c2a0a/12903_2024_5045_Fig6_HTML.jpg

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本文引用的文献

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Appl Immunohistochem Mol Morphol. 2019 Nov/Dec;27(10):740-748. doi: 10.1097/PAI.0000000000000683.
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Successful treatment of a rare subcutaneous panniculitis-like T-cell lymphoma: An unusual case report and literature review.罕见的皮下脂膜炎样T细胞淋巴瘤的成功治疗:一则罕见病例报告及文献综述
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胚系 HAVCR2 突变改变 TIM-3 特征性表现为伴有噬血细胞性淋巴组织细胞增生症的皮下脂膜炎样 T 细胞淋巴瘤。
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