Objective:To study the immunohistochemical characteristics of systemic anaplastic large cell lymphoma.Methods:A retrospective analysis of immunophenotype of 48 cases of systemic anaplastic large cell lymphoma and the result of EBV-encoded small RNA (EBER) expression with in situ hybridization of 10 cases was performed.Results:The tumor cells expressed CD30 in 48 cases ofALCL.Anaplastic lymphoma kinase (ALK)was positive in about 41.7％ (20/48) cases.The positive rates of other antigens were 65.0％ (26/40) of CD2,36.2％ (17/47) of CD3,72.7％ (16/22) of CD4,42.9％ (15/35) of CD5,16.7％ (5/30) of CD7,65.6％ (21/32)of epithelial membrane antigen (EMA),79.2％ (19/24) of TIA-1,and 70.0％ (14/20) of granzyme B-producing Breg (GrB).All of the cases were negative for B-cell markers (CD20,PAX-5 and CD79α).Two of 10 cases showed EBER positive in partial tumor cells.Conclusion:CD30 and ALK are the critical and relatively specific markers for ALCL;Epstein-Barr virus (EBV) involvement may not preclude the diagnosis of ALCL in some cases.%目的:探讨系统性间变性大细胞淋巴瘤(anaplastic large cell lymphoma,ALCL)的免疫组织化学特征.方法:回顾性分析48例系统性ALCL的免疫组织化学和10例系统性ALCL原位杂交技术检测EBER(EBV-encoded small RNA)的结果.结果:48例系统性ALCL的肿瘤细胞均表达CD30,而间变性淋巴瘤激酶(anaplastic lymphoma kinase,ALK)在41.7％(20/48)的病例阳性,其他指标阳性率为CD2为65.0％(26/40),CD3为36.2％(17/47),CD4为72.7％(16/22),CD5为42.9％(15/35),CD7为16.7％(5/30),上皮细胞膜抗原(epithelial membrane antigen,EMA)为65.6％(21/32),T细胞胞质内抗原(T-cell intracellular antigen-1,TIA-1)为79.2％(19/24),颗粒酶B(granzyme B-producing Breg,GrB)为70.0％(14/20).所有病例的B细胞标志(CD20,PAX5,CD79a)均阴性.10例系统性ALCL有2例出现部分肿瘤性大细胞EBER阳性.结论:CD30和ALK是诊断ALCL关键及较为特异的免疫指标;有时出现人类疱疹病毒第四型(Epstein-Barr virus,EBV)感染并不能排除ALCL的诊断.
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