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Human T-cell lymphotropic virus type-1 associated t-cell leukemia/lymphoma masquerading as necrotizing retinal vasculitis.

机译:伪装为坏死性视网膜血管炎的人类T细胞1型淋巴细胞性病毒相关的T细胞白血病/淋巴瘤。

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OBJECTIVE: To report a case of adult T-cell leukemia/lymphoma (ATL) presenting as a bilateral retinal vasculitis and diagnosed by molecular detection of a rearrangement in the T-cell receptor (TCR) and the presence of the human T-cell lymphotropic virus type 1 (HTLV-1) pol gene in the malignant lymphoid cells. DESIGN: Case report. METHODS: Routine histologic and immunohistochemical analyses were performed on the retinal biopsy specimen before referral to the National Eye Institute. Lymphoid cells associated with granulomatous inflammation infiltrating the retina and surrounding retinal blood vessels were microdissected from the paraffin sections of the retinal biopsy specimen. The polymerase chain reaction (PCR) was performed using primers for the TCR gene and HTLV-1 pol and gag genes. RESULTS: Microscopic examination showed a necrotizing granulomatous retinal vasculitis with a predominant T-cell infiltrate detected by immunohistochemistry. Molecular analysis demonstrated a clonal rearrangement of the TCR and the presence of the HTLV-1 pol gene in the microdissected lymphoid cells diagnostic of ATL. CONCLUSIONS: Necrotizing retinitis and retinal vasculitis are rare manifestations of ATL. Human T-cell lymphotropic virus type 1 infection should be considered in the differential diagnosis of patients from endemic areas who have retinal vasculitis at presentation. This case further demonstrates the usefulness of microdissection and PCR for the diagnosis of ocular disease, including HTLV-1 infection.
机译:目的:报告一例成人T细胞白血病/淋巴瘤(ATL),表现为双侧视网膜血管炎,并通过分子检测T细胞受体(TCR)的重排和人类T细胞淋巴细胞的存在进行诊断恶性淋巴样细胞中的病毒1型(HTLV-1)pol基因。设计:病例报告。方法:在转诊至美国国立眼科研究所之前,对视网膜活检标本进行了常规的组织学和免疫组织化学分析。从视网膜活检标本的石蜡切片中显微切割与浸润视网膜和周围视网膜血管的肉芽肿性炎症相关的淋巴样细胞。使用TCR基因和HTLV-1 pol和gag基因的引物进行聚合酶链反应(PCR)。结果:镜检可见坏死性肉芽肿性视网膜血管炎,免疫组织化学检测到主要的T细胞浸润。分子分析表明,TCR的克隆重排和HTLV-1 pol基因在诊断ATL的显微淋巴样细胞中存在。结论:坏死性视网膜炎和视网膜血管炎是ATL的罕见表现。在就诊时对来自地方性地区患有视网膜血管炎的患者进行鉴别诊断时,应考虑使用1型人类T细胞淋巴病毒。该病例进一步证明了显微解剖和PCR在眼疾病(包括HTLV-1感染)诊断中的有用性。

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