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伦内特淋巴瘤的临床病理诊断:病例报告及文献复习。

Clinicopathological diagnosis of Lennert lymphoma: a case report and review of the literature.

机构信息

Department of Pathology, Shenzhen Luohu People's Hospital, The Third Affiliated Hospital of Shenzhen University, Shenzhen, China.

Department of Hematology, Shenzhen Luohu People's Hospital, The Third Affiliated Hospital of Shenzhen University, Shenzhen, China.

出版信息

Diagn Pathol. 2024 Aug 16;19(1):111. doi: 10.1186/s13000-024-01533-x.

Abstract

BACKGROUND

Lennert lymphoma (LL) is a variant of peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS), also known as a lymphoepithelioid variant of PTCL. Because of the rarity and lack of clear-cut diagnostic criteria, LL is susceptible tomisdiagnosis. Although previously diagnosed with LL might be reclassified and evaluated with the advent of of molecular and/or genetic findings, cytomorphology and immunohistochemistry are still the key to give rise to correct diagnosis.

CASE PRESENTATION

We report a case of a patient who was diagnosed as LL based on cytomorphology and immunohistochemistry. Routine stain (Hematoxlin and Eosin-H&E) revealed tumor cells were mainly small to medium-sized CD4(+) T cells, the CD8 +/TIA-1 + cytotoxic cells were less minority, no expressions of follicle helper T cell markers (CD10, BCL6, PD1, CXCL13, ICOS) or CD21(+) hyperplastic FDC network, or proliferation of high edndothelial venules were noted; however, numerous epithelioid histiocytes are noted in the background and scattered EBV(+) cells were also present. The patient was achieved complete remission after six courses of chemotherapy with cyclophosphamide, epirubicin, vincristine, etoposide, and prednisone regimen. She was followed for 5 years without recurrence or progression.

CONCLUSIONS

Classic LL is not difficult to diagnose by cytomorphology and immunohistochemistry, and the mutation profiles can be helpful to distinguish LL from other lymphomas.

摘要

背景

Lennert 淋巴瘤(LL)是一种未特指的外周 T 细胞淋巴瘤(PTCL,NOS)的变体,也称为 PTCL 的淋巴上皮样变体。由于罕见且缺乏明确的诊断标准,LL 容易误诊。尽管随着分子和/或基因发现的出现,以前诊断为 LL 的病例可能会重新分类和评估,但细胞形态学和免疫组织化学仍然是做出正确诊断的关键。

病例介绍

我们报告了一例患者,根据细胞形态学和免疫组织化学诊断为 LL。常规染色(苏木精和伊红-H&E)显示肿瘤细胞主要为小至中等大小的 CD4(+)T 细胞,CD8+/TIA-1+细胞毒性细胞较少,滤泡辅助 T 细胞标志物(CD10、BCL6、PD1、CXCL13、ICOS)或 CD21(+)增生滤泡树突细胞网络无表达,或高内皮静脉无增生;然而,背景中存在大量上皮样组织细胞,并且还存在散在的 EBV(+)细胞。患者在接受环磷酰胺、表柔比星、长春新碱、依托泊苷和泼尼松方案的六个疗程化疗后达到完全缓解。她随访了 5 年,没有复发或进展。

结论

经典的 LL 通过细胞形态学和免疫组织化学不难诊断,突变谱有助于将 LL 与其他淋巴瘤区分开来。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/081f/11328506/d0587ec1e7e4/13000_2024_1533_Fig1_HTML.jpg

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