结节性淋巴细胞为主型霍奇金淋巴瘤伴少见免疫结构变异1例并文献复习  

Nodular lymphocyte-predominant Hodgkin lymphoma with rare immune structural varition: a case report and review of literature

作　　者：黄欣 赖玉梅[2] 刘校龙 刘翠苓 高子芬 HUANG Xin;LAI Yu-mei;LIU Xiao-long;LIU Cui-ling;GAO Zi-fen(Department of Pathology,School of Medical Sciences,Peking University Health Science Center /Peking University Third Hospital,Beijing100191,China;Department of Pathology,Peking University Cancer Hospital,Beijing100142,China)

机构地区：[1]北京大学医学部基础医学院病理系/北京大学第三医院病理科,北京100191 [2]北京大学肿瘤医院病理科,北京100142

出　　处：《临床与实验病理学杂志》2019年第5期529-534,共6页Chinese Journal of Clinical and Experimental Pathology

基　　金：国家自然科学基金(81201859)

摘　　要：目的观察结节性淋巴细胞为主型霍奇金淋巴瘤(nodular lymphocyte-predominant Hodgkin lymphoma,NLPHL)伴少见免疫结构变异即富于T细胞/组织细胞大B细胞淋巴瘤(T-cell/histiocyte-rich large B cell lymphoma,THRLBL)样转化的NLPHL的临床病理学特征,以提高对NLPHL免疫结构变异的认识、诊断及鉴别诊断。方法回顾性分析1例伴有THRLBL样转化的NLPHL的临床病理学特征及免疫表型。行EB病毒相关性和Ig/TCR基因克隆性检测,并复习相关文献。结果患者男性,58岁,腹股沟区无痛性淋巴结肿大。腹股沟淋巴结活检组织学观察可见淋巴结结构破坏,低倍镜下见浅染区和深染区交替分布,以浅染区为主,两种区域均可见散在分布的异型大细胞。免疫表型:大细胞一致强表达全B细胞标记(CD20、PAX5)、不表达CD30;CD21显示深染区内不规则滤泡树突细胞网结构,而浅染区内缺如。此外,两种结构背景细胞组成也存在明显差异。深染区背景细胞富于小B细胞,并可见PD1阳性细胞围绕大细胞形成花环样结构;浅染区背景细胞则以小T细胞和组织细胞为主,小B细胞基本缺如,且PD1阳性细胞量及强度均显著下降。EB病毒原位杂交检测两种结构内均无阳性细胞,Ig和TCR基因重排检测均未发现克隆性重排。结论伴有THRLBL样转化的NLPHL具有特殊形态学和免疫结构特征,易被误诊为原发性THRLBL,了解NLPHL免疫结构变异并结合细致全面的组织学观察和免疫组化检测有助于其诊断和鉴别诊断。Purpose To observe the clinicopathologic features of nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) with rare immune structural variation,that is,NLPHL with T-cell/histiocyte-rich large B cell lymphoma (THRLBL)-like transformation,and to improve the understanding,diagnosis and differential diagnosis of the variantion of immune structure of NLPHL. Methods The clinicopathologic features and immunotypes of a case of NLPHL with THRLBL-like transformation were retrospectively analysed,relevant literatures were also reviewed. Results The patient was a 58-year-old male who presented with painless inguinal lymphadenopathy. A diagnostic lymph node biopsy revealed the effacement of normal architecture and the alternative distribution of the light staining and the dark staining area at low magnification. Scattered large atypical cells could be seen in both areas. In immunophenotype,large cells uniformly and strongly expressed pan-B cell markers (CD20 and PAX5),and did not express CD30. CD21 staining highlighted irregular nodular growth pattern in the dark staining area,but not in the light staining area. In addition,there was significant differences in the composition of background cells between the two structures. The background cells in the dark staining area were rich in small B cells,and PD1 positive cells were found to form rosette-like structures around the large cells,while the background cells in the light staining area were mainly T cells and histiocytes,and the small B cells were almost absent. The number and intensity of PD1 positive cells decreased significantly. EB virus in situ hybridization showed no positive cells in both structures. Absence of TCR or Ig clonal rearrangement was demonstrated by PCR-based clonality assay. Conclusion NL- PHL with THRLBL-like transformation is a rare but unique im- munoarchitectural variant of NLPHL and a thorough evaluation of morphologic and immunophenotypic features is crucial for the diagnosis and differential diagnosis.

关 键 词：淋巴瘤 结节性淋巴细胞为主型霍奇金淋巴瘤 富于T细胞/组织细胞大B细胞淋巴瘤 免疫结构变异 滤泡树突细胞网 PD1 

分 类 号：R733.4[医药卫生—肿瘤]