临床与实验病理学杂志2024，Vol.40Issue(1)：72-76,5.DOI:10.13315/j.cnki.cjcep.2024.01.016
系统性ALK阴性间变性大细胞淋巴瘤的病理诊断
Pathological diagnosis of systemic ALK negative anaplastic large cell lymphoma
摘要
Abstract
Purpose To investigate the clinical and patho-logical characteristics,molecular characteristics,treatment and prognosis of systemic ALK-negative anaplastic large cell lympho-ma(ALCL).Methods Retrospective analysis was conducted on the clinical pathology,immunophenotype,molecular charac-teristics,treatment and prognosis of 18 cases of systemic ALK-ALCL.HE,immunohistochemistry,FISH,and NGS tests were performed,and relevant literatures were reviewed.Results Systemic ALK-ALCL tended to occur in elderly men,often in the advanced stage,mainly in lymph node lesions.The extran-odal primary sites included the primary pancreas and primary thoracic vertebrae.Morphological examination showed 17 cases belong to common type,1 case belong to"Hodgkin like"type.CD30 was diffuse and strongly positive in tumor cells(＞75％),CD2(16/17),CD3(13/18),CD5(4/18),CD7(8/18),CD4(14/18),TIA-1(16/18),CD8(2/16),GATA-3(10/12),EMA(3/5),MUM1(12/12),CD43(6/6)and CD56(2/8)were positive to varying degrees.The Ki67 proliferation index of 30％to 90％,PD-L1(22C3)(TPS=0-100％),ALK,CD15,CD79α and CD20 were all negative.FISH detection:5 cases of TP63 deficiency and 2 cases of DUSP22 deficiency;NGS detection:16 cases of gene mutations occurred,with a fre-quency of 0-11 gene mutations and an average of 4.2 gene mu-tations;ALK-ALCL with TP63 rearrangement was more likely to occur in women,mostly in lymph nodes,late clinical staging,susceptibility to p53 gene abnormalities,low PD-L1 expression rate and high mortality rate.Conclusion Systemic ALK-ALCL with TP63 rearrangement is associated with many adverse factors,the clinical process is often invasive with poor progno-sis.关键词
间变性大细胞淋巴瘤/ALK阴性/临床病理/TP63Key words
anaplastic large cell lymphoma/ALK negative/clinical pathology/TP63分类
医药卫生引用本文复制引用
程平,许海敏,张蕾,胡丽娟,李传应..系统性ALK阴性间变性大细胞淋巴瘤的病理诊断[J].临床与实验病理学杂志,2024,40(1):72-76,5.基金项目
安徽省自然科学基金面上项目(1808085MH286)、安徽中医药大学第一临床医学院临床自然科学重点项目(2021yfylc16) （1808085MH286）
