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一名意大利女性患成人T细胞白血病和淋巴瘤,其肿瘤肿块和血液中显示出不同的恶性克隆。

A case of adult T cell leukemia and lymphoma in an Italian woman showing different malignant clones in tumor mass and in blood.

作者信息

Menin Chiara, Bulian Pietro, Filippi Fausto, Buttarello Mauro, Casado Concepcion, Lopez-Galindez Cecilio, De Rossi Anita, Chieco-Bianchi Luigi, Del Mistro Annarosa

机构信息

Cytodiagnostic and Molecular Oncology Unit, Azienda Ospedaliera di Padova, via Gattamelata 64, 35128 Padova, Italy.

出版信息

Haematologica. 2003 Jul;88(7):ECR23.

Abstract

HTLV-1 infections and their associated diseases are very rare in Italy, as well as in most parts of Europe, occurring prevalently in subjects related to endemic areas. The HTLV-1-associated leukemia/lymphoma, ATLL, is a very aggressive T-cell non-Hodgkin's lymphoma which can be difficult to recognize in non-endemic areas. Here we describe the case of an elderly Italian woman, with no apparent risk factors, affected by a rapidly fatal ATLL who presented with an abdominal lymphomatous mass and circulating leukemic cells. The simultaneous presence of different T-cell clones in the tumor mass and in the blood was demonstrated by T-cell receptor gene rearrangement analysis and HTLV-1 integration pattern studies. After surgery, all the T-cell clones were present in the blood, indicating that tumor cells had spread from the mass. Phylogenetic analysis, using the complete LTR sequence, showed that the patient's HTLV-1 isolate belongs to the cosmopolitan subtype A.

摘要

人类嗜T淋巴细胞病毒1型(HTLV-1)感染及其相关疾病在意大利以及欧洲大部分地区都非常罕见,主要发生在与流行地区相关的人群中。HTLV-1相关白血病/淋巴瘤(ATLL)是一种侵袭性很强的T细胞非霍奇金淋巴瘤,在非流行地区可能难以识别。在此,我们描述了一名老年意大利女性的病例,她没有明显的危险因素,却患上了迅速致命的ATLL,表现为腹部淋巴瘤肿块和循环白血病细胞。通过T细胞受体基因重排分析和HTLV-1整合模式研究,证实肿瘤肿块和血液中同时存在不同的T细胞克隆。手术后,所有T细胞克隆都出现在血液中,表明肿瘤细胞已从肿块扩散。使用完整的长末端重复序列(LTR)进行系统发育分析表明,患者的HTLV-1分离株属于世界性A亚型。

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