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在一名来自成人T细胞白血病/淋巴瘤(ATLL)流行地区的患者中,同时出现爱泼斯坦-巴尔病毒相关霍奇金淋巴瘤和成人T细胞白血病/淋巴瘤(ATLL)。

Synchronous presentation of Epstein-Barr virus-associated Hodgkin's disease and adult T-cell leukemia/lymphoma (ATLL) in a patient from an endemic area of ATLL.

作者信息

Hayashi T, Yamabe H, Haga H, Akasaka T, Kadowaki N, Ohno H, Okuma M, Fukuhara S

机构信息

Department of Internal Medicine, Kyoto University, Japan.

出版信息

Int J Hematol. 1995 Jun;61(4):215-22. doi: 10.1016/0925-5710(95)00365-y.

DOI:10.1016/0925-5710(95)00365-y
PMID:8547610
Abstract

We report a patient from an endemic area of adult T-cell leukemia/lymphoma (ATLL), who developed lymphoma with features characteristic of Hodgkin's disease (HD). Large atypical Reed-Sternberg/Hodgkin's cells (RS/H cells) had a CD3-CD15+CD20-CD30+CD45RO- immunophenotype. Epstein-Barr virus (EBV) latent membrane protein and EBV-encoded small RNA were detected in the RS/H cells. The patient received C-MOPP/ABVD chemotherapy for the HD resulting in a partial response. However, relapse occurred and he died of disease progression associated with serious bacterial infection. Although serial lymph node biopsies revealed consistent presence of the EBV-positive RS/H cells, the background small lymphocytes showed progressive increase in pleomorphism and nuclear irregularity. The lymphocytes had the T-cell phenotype, CD3+CD4+CD7-CD8-. Southern blot analysis using DNA probes for the human T-cell lymphotrophic virus-I (HTLV-I) and the T-cell receptor beta-chain gene demonstrated expansion of the HTLV-I infected monoclonal T-cells with the disease progression. We concluded that the patient synchronously presented two independent lymphoproliferative disorders; EBV-associated HD and ATLL resulting from HTLV-I infection.

摘要

我们报告了一名来自成人T细胞白血病/淋巴瘤(ATLL)流行地区的患者,该患者发生了具有霍奇金病(HD)特征的淋巴瘤。大型非典型里德-斯腾伯格/霍奇金细胞(RS/H细胞)具有CD3-CD15+CD20-CD30+CD45RO-免疫表型。在RS/H细胞中检测到爱泼斯坦-巴尔病毒(EBV)潜伏膜蛋白和EBV编码的小RNA。该患者接受了针对HD的C-MOPP/ABVD化疗,结果为部分缓解。然而,疾病复发,他死于与严重细菌感染相关的疾病进展。尽管系列淋巴结活检显示EBV阳性RS/H细胞持续存在,但背景小淋巴细胞的多形性和核不规则性逐渐增加。这些淋巴细胞具有T细胞表型,CD3+CD4+CD7-CD8-。使用针对人类T细胞嗜淋巴细胞病毒-I(HTLV-I)和T细胞受体β链基因的DNA探针进行的Southern印迹分析表明,随着疾病进展,HTLV-I感染的单克隆T细胞发生扩增。我们得出结论,该患者同时出现了两种独立的淋巴增殖性疾病;与EBV相关的HD和由HTLV-I感染引起的ATLL。

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