Updates in lymph node and skin pathology of adult T-cell leukemia/lymphoma, biomarkers, and beyond.

Updates in lymph node and skin pathology of adult T-cell leukemia/lymphoma, biomarkers, and beyond.
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DOI:
10.1053/j.semdp.2019.12.006
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发表时间:
2020-01
影响因子:
2.3
通讯作者:
Gru AA
Gru AA
中科院分区:
医学3区
文献类型:
--
作者:
Adkins BD;Ramos JC;Bliss-Moreau M;Gru AA

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成人T细胞白血病/淋巴瘤(ATLL)是一种与人类嗜T淋巴细胞病毒(HTLV-1)感染相关的成熟T细胞淋巴增生性疾病。ATLL主要影响HTLV-1流行地区的个体,如日本、非洲、南美洲和加勒比地区。HTLV-1优先感染CD 4 + T细胞,并且在ATLL发展之前必须发生几次遗传命中。ATLL根据疾病的表现分为四种临床变异:急性、慢性、淋巴瘤和阴燃。截至2019年,已描述了一种新的亚型:ATL淋巴瘤型,淋巴结原发性皮肤。在这篇综述中,重点将描述该疾病的常见临床病理表现、生物标志物发现的进展、突变情况和治疗这种高度侵袭性且经常致命的T细胞淋巴瘤的靶向治疗方法。
Adult T-cell leukemia/lymphoma (ATLL) is a mature T-cell lymphoproliferative disorder associated with the human T lymphotropic virus (HTLV-1) infection. ATLL predominantly affects individuals within HTLV-1 endemic areas such as Japan, areas of Africa, South America, and the Caribbean. HTLV-1 preferentially infects CD4+ T-cells, and several genetic hits must occur before ATLL develops. ATLL is classically divided into four clinical variants based on manifestations of disease: acute, chronic, lymphomatous, and smouldering. As of 2019, a new subtype has been described: lymphoma type of ATL, extranodal primary cutaneous. In this review, emphasis will be taken to describe the common clinicopathologic manifestations of the disease, advances in biomarker discovery, mutational landscape and targeted therapeutic approaches to treat this highly aggressive and frequently lethal type of T-cell lymphoma.
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