关键词: Case report Epithelioid histiocytes Lennert lymphoma PTCL-NOS RS-like cells

Mesh : Humans Immunohistochemistry Biomarkers, Tumor / analysis Lymphoma, T-Cell, Peripheral / pathology diagnosis Antineoplastic Combined Chemotherapy Protocols / therapeutic use Female Middle Aged Treatment Outcome Predictive Value of Tests

来  源:   DOI:10.1186/s13000-024-01533-x   PDF(Pubmed)

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.
METHODS: 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)是外周T细胞淋巴瘤的一种变体,未指定(PTCL,NOS),也被称为PTCL的淋巴上皮样变体。由于罕见且缺乏明确的诊断标准,LL是敏感的诊断。尽管先前诊断为LL可能会随着分子和/或遗传发现的出现而重新分类和评估,细胞形态学和免疫组织化学仍然是引起正确诊断的关键。
方法:我们报告一例患者,根据细胞形态学和免疫组织化学诊断为LL。常规染色(Hematoxlin和Eosin-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,滤泡辅助性T细胞标志物(CD10,BCL6,PD1,CXCL13,ICOS)或CD21(+)增生性FDC网络无表达,或高内皮小静脉的增殖被注意到;然而,背景中注意到许多上皮样组织细胞,并且还存在分散的EBV(+)细胞。患者在环磷酰胺化疗6个疗程后达到完全缓解,表柔比星,长春新碱,依托泊苷,和泼尼松方案。随访5年,无复发或进展。
结论:经典LL不难通过细胞形态学和免疫组织化学来诊断,突变谱有助于区分LL和其他淋巴瘤。
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