Liu Melissa M, Furusato Emiko, Cao Xiaoguang, Shen Defen, Chan Chi-Chao
Immunopathology Section, Laboratory of Immunology, National Eye Institute, National Institutes of Health, Bethesda, Maryland, USA.
Rare Tumors. 2010 Dec 31;2(4):e63. doi: 10.4081/rt.2010.e63.
The human T-cell lymphotropic virus type 1 (HTLV-1), endemic in defined geographical areas around the world, is recognized as the etiologic agent of adult T-cell leukemia/lymphoma (ATL), or HTLV-1. ATL is a rare adult onset T-cell malignancy that is characterized by the presence of ATL flower cells with T-cell markers, HTLV-1 antibodies in the serum, and monoclonal integration of HTLV-1 provirus in affected cells. Ocular manifestations associated with HTLV-1 virus infection have been reported and include HTLV-1 uveitis and keratoconjunctivitis sicca, but reports of ocular involvement in ATL are exceedingly rare. This article describes the ocular manifestations and pathology of ATL. We also report for the first time a case of a 34-year-old male with systemic ATL and prominent atypical lymphoid cell infiltration in the choroid. To our knowledge, this is the first report defining prominent choroidal involvement as a distinct ocular manifestation of ATL. ATL may masquerade as a variety of other conditions, and molecular techniques involving microdissection and PCR have proven to be critical diagnostic tools. International collaboration will be needed to better understand the presentation and diagnosis of this rare malignancy.
人类嗜T细胞病毒1型（HTLV-1）在世界上某些特定地理区域呈地方性流行，被公认为是成人T细胞白血病/淋巴瘤（ATL）或HTLV-1相关疾病的病原体。ATL是一种罕见的成人发病的T细胞恶性肿瘤，其特征是存在带有T细胞标志物的ATL花细胞、血清中的HTLV-1抗体，以及受影响细胞中HTLV-1前病毒的单克隆整合。与HTLV-1病毒感染相关的眼部表现已有报道，包括HTLV-1葡萄膜炎和干燥性角结膜炎，但ATL累及眼部的报道极为罕见。本文描述了ATL的眼部表现和病理学。我们还首次报告了一例34岁男性全身性ATL患者，其脉络膜有明显的非典型淋巴细胞浸润。据我们所知，这是首次将明显的脉络膜受累定义为ATL一种独特的眼部表现的报告。ATL可能会伪装成多种其他疾病，涉及显微切割和PCR的分子技术已被证明是关键的诊断工具。需要国际合作来更好地了解这种罕见恶性肿瘤的表现和诊断。
