富t细胞霍奇金淋巴瘤具有经典霍奇金淋巴瘤和结节淋巴细胞为主的霍奇金淋巴瘤的特征:一个具有重叠形态学和免疫表型特征的边缘类别。

Siba El Hussein, Hong Fang, Fatima Zahra Jelloul, Wei Wang, Sanam Loghavi, Roberto N Miranda, Jonathan W Friedberg, W Richard Burack, Andrew G Evans, Jie Xu, L Jeffrey Medeiros
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引用次数: 0

摘要

上下文。-:我们知道,一部分典型霍奇金淋巴瘤(CHL)伴有富b细胞结节(富淋巴细胞CHL),其形态学和免疫表型特征与结节性淋巴细胞为主的霍奇金淋巴瘤(NLPHL)重叠,这增加了诊断困难,在大多数病例中,通过进行充分的免疫组织化学研究可以解决。-:充分表征富t细胞霍奇金淋巴瘤的病例,即使在完整的免疫表型调查后也不能做出NLPHL(即D型)或CHL的特异性诊断。研究了3例富t细胞霍奇金淋巴瘤的临床、免疫形态学和分子(如适用)表现。-:这3例患者具有淋巴细胞为主样细胞和霍奇金细胞和里德-斯特恩伯格细胞样细胞,这些细胞部分表达b细胞和CHL标志物,Epstein-Barr病毒编码的小RNA呈阴性,在t细胞丰富的背景下残留滤泡树突状细胞网;嗜酸性粒细胞和浆细胞少/无2例,多见1例。2例晚期(III或IV)患者分别表现为腋窝和锁骨上淋巴结病变,无B症状。这些患者接受了nlphl样的治疗管理,包括6个周期的R-CHOP(利妥昔单抗、环磷酰胺、盐酸阿霉素[羟基柔红霉素]、硫酸长春新碱[Oncovin]和强的松)化疗;两人均在治疗后7年完全缓解。1例患者出现I期疾病,累及乳腺内淋巴结,无b症状,仅行手术切除治疗;该患者在1年后也完全缓解。-:这些病例说明了富含t细胞的NLPHL和CHL与肿瘤细胞同时表达b细胞程序和CHL标记物的重叠特征。这种未被充分认识的重叠在文献中尚未得到充分说明,尽管它描绘了治疗上的挑战。这些肿瘤可能值得在未来进行深入的研究,以提出诊断或治疗意义。
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T-Cell-Rich Hodgkin Lymphoma With Features of Classic Hodgkin Lymphoma and Nodular Lymphocyte-Predominant Hodgkin Lymphoma: A Borderline Category With Overlapping Morphologic and Immunophenotypic Features.

Context.—: It is known that a subset of cases of classic Hodgkin lymphoma (CHL) with B-cell-rich nodules (lymphocyte-rich CHL) exhibits morphologic and immunophenotypic features that overlap with nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL), raising diagnostic difficulties that can be resolved in most cases by performing an adequate battery of immunohistochemical studies.

Objective.—: To fully characterize cases of T-cell-rich Hodgkin lymphoma where a specific diagnosis of NLPHL (ie, pattern D) or CHL could not be made even after complete immunophenotypic investigation.

Design.—: The clinical, immunomorphologic, and molecular (when applicable) presentation of 3 cases of T-cell-rich Hodgkin lymphoma was thoroughly investigated.

Results.—: These 3 cases harbored lymphocyte-predominant-like and Hodgkin and Reed-Sternberg-like cells that partially expressed B-cell and CHL markers and were negative for Tiftein-Barr virus-encoded small RNA, in a T-cell-rich background with residual follicular dendritic cell meshworks; 1 case had frequent and the other 2 cases scant/absent eosinophils and plasma cells. Two patients with advanced-stage (III or IV) disease presented with axillary and supraclavicular lymphadenopathy, respectively, and without B symptoms. These patients underwent NLPHL-like therapeutic management with 6 cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin hydrochloride [hydroxydaunorubicin], vincristine sulfate [Oncovin], and prednisone) chemotherapy; both are in complete remission 7 years posttherapy. One patient presented with stage I disease involving an internal mammary lymph node without B-symptoms and was treated with surgical excision alone; this patient is also in complete remission 1 year later.

Conclusions.—: These cases illustrate overlapping features of T-cell-rich NLPHL and CHL with neoplastic cells expressing both B-cell program and CHL markers. This underrecognized overlap has not been fully illustrated in the literature, although it portrays a therapeutic challenge. These neoplasms may deserve in-depth investigation in the future that may bring up diagnostic or theragnostic implications.

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