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儿童种痘水疱病样皮肤T细胞淋巴瘤:一例报告

Hydroa vacciniforme-like cutaneous T-cell lymphoma in a child: A case report.

作者信息

Li Jingyi, Zan Yiheng, Liu Hongjie, Liu Hanmin, Chen Lina

机构信息

Department of Pediatrics, West China Second University Hospital Key Laboratory of Birth Defects and Related Diseases of Women and Children, Ministry of Education Department of Dermatology, West China Hospital, Sichuan University, Chengdu, Sichuan, People's Republic of China.

出版信息

Medicine (Baltimore). 2018 Apr;97(15):e0319. doi: 10.1097/MD.0000000000010319.

Abstract

RATIONALE

Hydroa vacciniforme (HV)-like T-cell lymphoma is a rare malignancy in childhood associated with Epstein-Barr virus infection.

PATIENT CONCERNS

A 6-year old girl presented with complaint of 3-year history of recurrent skin lesions, 3 months of fever accompanied by cough for 8 days.

DIAGNOSES

Skin biopsy revealed a HV-like lymphoma presentation and positive signals of EBER were detected by in situ hybridization. TCR-γ gene monoclonal rearrangement was present. A HV-like cutaneous T-cell lymphoma was diagnosed.

INTERVENTIONS

The girl was treated with cyclosporine and CHOP.

OUTCOMES

The girl's condition had been stable for 6 months.

LESSONS

Our case highlights the necessity for taking the HV-like lymphoma as a differential diagnosis especially when a patient manifests as recurrent skin lesions accompanied by systemic involvement.

摘要

原理

种痘样水疱病(HV)样T细胞淋巴瘤是一种与爱泼斯坦-巴尔病毒感染相关的儿童罕见恶性肿瘤。

患者情况

一名6岁女孩,有3年复发性皮肤病变病史,发热3个月,伴有咳嗽8天。

诊断

皮肤活检显示为HV样淋巴瘤表现,原位杂交检测到EBER阳性信号。存在TCR-γ基因单克隆重排。诊断为HV样皮肤T细胞淋巴瘤。

干预措施

该女孩接受了环孢素和CHOP治疗。

结果

女孩病情稳定6个月。

经验教训

我们的病例强调了将HV样淋巴瘤作为鉴别诊断的必要性,尤其是当患者表现为复发性皮肤病变并伴有全身受累时。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7da5/5908608/e915eefab3ac/medi-97-e0319-g001.jpg

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