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秘鲁一家医院中1型人类嗜T淋巴细胞病毒(HTLV-1)阳性患者的成人T细胞白血病/淋巴瘤

Adult T-cell leukemia/lymphoma in a Peruvian hospital in human T-lymphotropic virus type 1 (HTLV-1) positive patients.

作者信息

Rodríguez-Zúñiga Milton José Max, Cortez-Franco Florencio, Qujiano-Gomero Eberth

机构信息

Dermatology Department, Hospital Nacional Daniel Alcides Carrion, Callao, Peru.

Universidad Nacional Mayor de San Marcos, Lima, Peru.

出版信息

Int J Dermatol. 2017 May;56(5):503-509. doi: 10.1111/ijd.13567. Epub 2017 Mar 2.

Abstract

BACKGROUND

Adult T-cell leukemia/lymphoma (ATLL) is an aggressive neoplasm of T-lymphocytes associated with human T-lymphotropic virus type I (HTLV-1) infection. As HTLV-1 is endemic in native ethnics in South America, and its infection leads to several chronic diseases as ATLL with poor prognosis, we aimed to present three ATLL cases and to review current literature.

CASE REPORTS

Two cases were from the mountains of Peru, while one was from an endemic harbor of the country. An acute ATLL patient presented with multipapular infiltration of the skin and died 2 weeks after admission because of septic shock. The two chronic ATLL patients presented with erythematous plaques and erythroderma. They had swollen lymph nodes, lymphocytosis, and atypical lymphocytes on blood smear, with normal biochemical results. They both passed away a few months after diagnosis.

COMMENTS

ATLL is developed after years of HTLV-1 carrier status; therefore, physicians should know the principal clinical and laboratory findings in order to make prompt diagnosis. Prognosis is still poor in aggressive and indolent variants, with survival rates from months to a few years. Treatment based on chemotherapy, antiretroviral, and allogeneic stem cell transplantation are improving survival rates but with limited results.

摘要

背景

成人T细胞白血病/淋巴瘤(ATLL)是一种与I型人类嗜T淋巴细胞病毒(HTLV-1)感染相关的侵袭性T淋巴细胞肿瘤。由于HTLV-1在南美洲的原住民中呈地方性流行,且其感染会导致包括预后较差的ATLL在内的多种慢性疾病,我们旨在介绍3例ATLL病例并回顾当前文献。

病例报告

2例来自秘鲁山区,1例来自该国的一个地方性流行地区。1例急性ATLL患者表现为皮肤多发性丘疹浸润,入院后2周因感染性休克死亡。2例慢性ATLL患者表现为红斑性斑块和红皮病。他们有淋巴结肿大、淋巴细胞增多,血涂片可见非典型淋巴细胞,生化检查结果正常。他们在诊断后几个月均去世。

评论

ATLL是在多年的HTLV-1携带者状态后发生的;因此,医生应了解主要的临床和实验室检查结果以便做出及时诊断。侵袭性和惰性亚型的预后仍然很差,生存率从数月到数年不等。基于化疗、抗逆转录病毒治疗和异基因干细胞移植的治疗正在提高生存率,但效果有限。

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