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原发性皮肤冒烟型成人T细胞白血病/淋巴瘤

Primary cutaneous smoldering adult T-cell leukemia/ lymphoma.

作者信息

Gittler Julia, Martires Kathryn, Terushkin Vitaly, Brinster Nooshin, Ramsay David

机构信息

Ronald O. Perelman Department of Dermatology, NYU School of Medicine, NYU Langone Medical Center.

出版信息

Dermatol Online J. 2016 Dec 15;22(12):13030/qt14d1b851.

Abstract

HTLV-1 is a virus that is endemic in southwesternJapan and the Caribbean and has been implicatedin the development of ATLL. ATLL, which is anuncommon malignant condition of peripheralT-lymphocytes, is characterized by four clinicalsubtypes, which include acute, lymphomatous,chronic, and smoldering types, that are based onLDH levels, calcium levels, and extent of organinvolvement. We present a 52-year- old woman withpruritic patches with scale on the buttocks and withtender, hyperpigmented macules and papules oftwo-years duration. Histopathologic examinationwas suggestive of mycosis fungoides, laboratoryresults showed HTLV-I and II, and the patient wasdiagnosed with primary cutaneous ATLL. We reviewthe literature on HTLV-1 and ATLL and specifically theprognosis of cutaneous ATLL. The literature suggeststhat a diagnosis of ATLL should be considered amongpatients of Caribbean origin or other endemicareas with skin lesions that suggest a cutaneousT-cell lymphoma, with clinicopathologic features ofmycosis fungoides. Differentiation between ATLLand cutaneous T-cell lymphoma is imperative as theyhave different prognoses and treatment approaches.

摘要

人类嗜T淋巴细胞病毒1型(HTLV-1)是一种在日本西南部和加勒比地区流行的病毒,与成人T细胞白血病淋巴瘤(ATLL)的发生有关。ATLL是一种外周T淋巴细胞的罕见恶性疾病,其特征有四种临床亚型,包括急性、淋巴瘤型、慢性和潜伏型,这些亚型是根据乳酸脱氢酶水平、钙水平和器官受累程度划分的。我们报告一名52岁女性,臀部有瘙痒性鳞屑斑块,伴有持续两年的压痛性色素沉着斑和丘疹。组织病理学检查提示蕈样肉芽肿,实验室结果显示HTLV-I和II阳性,该患者被诊断为原发性皮肤ATLL。我们回顾了关于HTLV-1和ATLL的文献,特别是皮肤ATLL的预后。文献表明,对于来自加勒比地区或其他流行地区、有提示皮肤T细胞淋巴瘤的皮肤病变且具有蕈样肉芽肿临床病理特征的患者,应考虑诊断为ATLL。区分ATLL和皮肤T细胞淋巴瘤至关重要,因为它们的预后和治疗方法不同。

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