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淋巴瘤型成人T细胞白血病-淋巴瘤,伴有巨大皮肤肿瘤,显示多个人类T淋巴细胞病毒1型DNA整合。

Lymphoma-type adult T-cell leukaemia-lymphoma with a bulky cutaneous tumour showing multiple human T-lymphotropic virus-1 DNA integration.

作者信息

Kato N, Sugawara H, Aoyagi S, Mayuzumi M

机构信息

Department of Dermatology and Clinical Research Institute, National Sapporo Hospital, Kikusui 4-2, Shiroishi-ku, Sapporo 003-0804, Japan.

出版信息

Br J Dermatol. 2001 Jun;144(6):1244-8. doi: 10.1046/j.1365-2133.2001.04242.x.

Abstract

Human T-lymphotropic virus-1 (HTLV-1) is considered to be the cause of adult T-cell leukaemia-lymphoma (ATL). Monoclonal integration of HTLV-1 proviral DNA, as is analysed by Southern blotting, has been demonstrated in ATL patients. Unusual integration patterns of HTLV-1 proviral DNA have occasionally been described, and it is suggested that the patterns have clinical implications for ATL pathophysiology. Multiple, complete and defective types of integration patterns, in that order, are apparently associated with prognoses from good to poor. We report a 73-year-old Japanese woman with lymphoma-type ATL and a bulky cutaneous tumour on the left thigh. Four bands of slightly differing intensity were seen after EcoRI digestion of skin and lymph node samples on Southern blot analysis of HTLV-1 proviral DNA. Analysis for T-cell receptor-beta gene revealed five novel bands after restriction enzyme digestion with HindIII, indicating that the patient has four separate tumour cell clones, each of which carries one copy of the provirus. She was treated with chemotherapy and radiation and remains under reasonable control despite some relapsing cutaneous nodules. The indolent course in this present case could be related to the multiple integration pattern of HTLV-1 proviral DNA detected.

摘要

人类嗜T淋巴细胞病毒1型(HTLV-1)被认为是成人T细胞白血病淋巴瘤(ATL)的病因。通过Southern印迹分析,已在ATL患者中证实了HTLV-1前病毒DNA的单克隆整合。偶尔会描述HTLV-1前病毒DNA的异常整合模式,并且提示这些模式对ATL病理生理学具有临床意义。多种、完整和缺陷类型的整合模式,依次明显与从良好到不良的预后相关。我们报告了一名73岁的日本女性,患有淋巴瘤型ATL,左大腿有一个巨大的皮肤肿瘤。对HTLV-1前病毒DNA进行Southern印迹分析时,在对皮肤和淋巴结样本进行EcoRI消化后,可见四条强度略有不同的条带。对T细胞受体β基因的分析显示,在用HindIII进行限制性酶切后出现了五条新条带,表明该患者有四个独立的肿瘤细胞克隆,每个克隆携带一份前病毒。她接受了化疗和放疗,尽管有一些皮肤结节复发,但病情仍得到合理控制。本病例的惰性病程可能与检测到的HTLV-1前病毒DNA的多重整合模式有关。

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