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淋巴细胞为主型霍奇金淋巴瘤与T细胞淋巴瘤并存。三例报告。

Concurrent lymphocyte predominance Hodgkin's disease and T-cell lymphoma. A report of three cases.

作者信息

Delabie J, Greiner T C, Chan W C, Weisenburger D D

机构信息

Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha 69198-3135, USA.

出版信息

Am J Surg Pathol. 1996 Mar;20(3):355-62. doi: 10.1097/00000478-199603000-00013.

Abstract

Lymphocyte predominance Hodgkin's disease (LPHD) is a B-cell lymphoproliferative disorder; patients with LPHD have an increased risk of developing synchronous or metachronous B-cell non-Hodgkin's lymphoma. The synchronous presence of LPHD and B-cell lymphoma in the same lymph node in some cases lends support to the argument that the B-cell lymphoma arises as a consequence of transformation or progression of LPHD. We have recently identified three cases of LPHD occurring simultaneously with T-cell lymphoma in a series of 76 cases of LPHD in the files of the Nebraska Lymphoma Study Group Registry. In large areas of the lymph nodes, atypical T cells with large, irregular, and hyperchromatic nuclei were admixed with Reed-Sternberg variants characteristic of LPHD (L&H cells). However, in all cases, areas of typical nodular LPHD without obvious T-cell lymphoma were also evident. In one case, frozen-section immunohistochemistry demonstrated the absence of expression of CD5, CD4, or CD8 by the T-cell lymphoma. The L&H cells in all cases expressed CD45 and CD20, as expected. In all three cases, clonal T-cell receptor (TCR)-gamma gene and TCR-beta gene rearrangements were documented by polymerase chain reaction analysis and Southern blotting, respectively. No clonally rearranged immunoglobulin genes were detected by either technique. To our knowledge, this represents the first report of the simultaneous occurrence of LPHD and T-cell lymphoma. Although B-cell lymphoma occurring in the setting of LPHD is a well-recognized phenomenon, previous reports of T-cell lymphoma occurring after a diagnosis of LPHD, as well as our cases with synchronous disease, suggest that the association of T-cell lymphoma and LPHD may not be uncommon as well. Furthermore, our cases indicate that T-cell lymphoma occurring in LPHD is not therapy related. However, the underlying mechanisms by which these composite lymphomas occur remain unknown.

摘要

淋巴细胞为主型霍奇金病(LPHD)是一种B细胞淋巴增殖性疾病;LPHD患者发生同步或异时性B细胞非霍奇金淋巴瘤的风险增加。在某些情况下,LPHD和B细胞淋巴瘤在同一淋巴结中同时存在,这支持了B细胞淋巴瘤是LPHD转化或进展的结果这一观点。我们最近在内布拉斯加淋巴瘤研究组登记处的76例LPHD病例中,发现了3例与T细胞淋巴瘤同时发生的LPHD病例。在淋巴结的大片区域,具有大的、不规则的和深染核的非典型T细胞与LPHD特征性的里德-斯腾伯格变异细胞(L&H细胞)混合存在。然而,在所有病例中,也都有典型的结节性LPHD区域,且无明显的T细胞淋巴瘤。在1例病例中,冰冻切片免疫组化显示T细胞淋巴瘤不表达CD5、CD4或CD8。所有病例中的L&H细胞均如预期表达CD45和CD20。在所有3例病例中,分别通过聚合酶链反应分析和Southern印迹法记录了克隆性T细胞受体(TCR)-γ基因和TCR-β基因重排。两种技术均未检测到克隆性重排的免疫球蛋白基因。据我们所知,这是LPHD与T细胞淋巴瘤同时发生的首次报告。虽然LPHD背景下发生B细胞淋巴瘤是一种公认的现象,但先前关于LPHD诊断后发生T细胞淋巴瘤的报告以及我们的同步疾病病例表明,T细胞淋巴瘤与LPHD的关联可能也并不罕见。此外,我们的病例表明,LPHD中发生的T细胞淋巴瘤与治疗无关。然而,这些复合淋巴瘤发生的潜在机制仍然未知。

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