首页|期刊导航|临床与实验病理学杂志|T-幼淋巴细胞白血病在淋巴结穿刺活检中的诊断特征

T-幼淋巴细胞白血病在淋巴结穿刺活检中的诊断特征OA

Diagnostic features of T-prolymphocytic leukemia in lymph node core needle bi-opsy specimens

中文摘要英文摘要

目的 探讨T-幼淋巴细胞白血病(T-prolymphocytic leukemia,T-PLL)在淋巴结穿刺活检中的诊断特征及其与其他成熟T/NK细胞白血病/淋巴瘤的鉴别诊断.方法 收集5例T-PLL患者的临床资料及病理标本.免疫组化采用EnVision两步法进行染色,EBER原位杂交检测EB病毒感染状态,PCR-毛细管电泳法检测TCR基因重排,流式细胞术进行免疫分型,RHG显带技术进行染色体核型分析,并针对84个T细胞淋巴瘤相关基因进行下一代测序(next-generations sequencing,NGS)检测.结果 淋巴结穿刺活检中,T-PLL具有稳定的形态学特征:肿瘤细胞染色质中等细腻;肿瘤细胞围绕血管呈"洋葱皮样"浸润;残存B细胞呈"岛屿状"分布.免疫表型:肿瘤细胞表达CD2、CD3、CD5、CD7、TCL1A及CD52,CD4/CD8呈两种表达模式.EBER原位杂交4例阴性,1例仅个别细胞阳性.5例骨髓流式细胞术均检出异常成熟T细胞克隆,免疫表型与组织学结果一致.5例均检出TCR基因克隆性重排,4例完成染色体核型分析,其中2例存在复杂核型异常.4例接受靶向NGS检测,检出ATM、JAK3、STAT5B、NOTCH1等基因体细胞突变.结论 T-PLL在淋巴结穿刺活检中的三项特征性形态学表现,有助于病理医师在面临淋巴结活检的疑难病例时把握诊断方向,减少误诊.

Objective To investigate the diagnostic features of T-prolymphocytic leukemia(T-PLL)in lymph node core needle biopsy specimens and its differential diagnosis from other mature T/NK-cell leukemias/lymphomas.Methods Clinical data and pathological specimens from five patients with T-PLL were collected.Immunohistochem-istry was performed using the EnVision two-step method.Epstein-Barr virus infection status was assessed by EBER in situ hybridization.T-cell receptor(TCR)gene rearrangements were analyzed by polymerase chain reaction followed by capillary electrophoresis.Immunophenotyping was performed by flow cytometry,and conventional cytogenetic analysis was performed using RHG banding.Targeted next-generation sequencing(NGS)of 84 T-cell lymphoma-related genes was also performed.Results Three reproducible morphologic features were identified in lymph node core needle bi-opsy specimens of T-PLL:tumor cells had moderately condensed,finely textured chromatin;tumor cells formed con-centric perivascular layers with an onion-skin appearance;and residual B cells showed an island-like distribution.The tumor cells expressed CD2,CD3,CD5,CD7,TCL1A,and CD52 and showed two CD4/CD8 expression patterns.EBER in situ hybridization was negative in four cases,whereas only scattered cells were positive in one case.Flow cy-tometry detected an abnormal mature T-cell clone in the bone marrow of all five patients,and the immunophenotypic findings were consistent with the histologic findings.Clonal TCR gene rearrangements were detected in all five cases.Cytogenetic analysis was completed in four cases,two of which showed complex karyotypic abnormalities.Targeted NGS was performed in four cases and identified somatic mutations in genes including ATM,JAK3,STAT5B,and NOTCH1.Conclusion The three characteristic morphologic features of T-PLL in lymph node core needle biopsy specimens may help pathologists recognize this entity in challenging lymph node biopsy cases and reduce misdiagnosis.

李琴;易红梅;李安琪;王朝夫;欧阳斌燊

成都市第五人民医院·成都中医药大学附属第五人民医院病理科,成都 611130上海交通大学医学院附属瑞金医院病理科,上海 200025上海交通大学医学院附属瑞金医院病理科,上海 200025上海交通大学医学院附属瑞金医院病理科,上海 200025上海交通大学医学院附属瑞金医院病理科,上海 200025

医药卫生

T-幼淋巴细胞白血病淋巴结穿刺病理诊断形态学鉴别诊断

T-prolymphocytic leukemialymph node core needle biopsypathological diagnosismorphologydiffer-ential diagnosis

《临床与实验病理学杂志》 2026 (7)

873-880,8

10.13315/j.cnki.cjcep.2026.07.005

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