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成人水疱样疹样淋巴增生性疾病侵犯肝脏和骨髓,有明确的病理证据:病例报告及文献复习。

Hydroa Vacciniforme-like Lymphoproliferative disorder in an adult invades the liver and bone marrow with clear pathological evidence: a case report and literature review.

机构信息

Center of Infectious Diseases, Beijing Ditan Hospital, Capital Medical University, 8 Jingshun East Street, Chaoyang District, Beijing, 100015, China.

Department of Pathology, Beijing Ditan Hospital, Capital Medical University, Beijing, China.

出版信息

BMC Infect Dis. 2021 Jan 6;21(1):17. doi: 10.1186/s12879-020-05697-x.

Abstract

BACKGROUND

Hydroa Vacciniforme-like Lymphoproliferative Disorder (HV-LPD) is the name given to a group of Epstein-Barr virus (EBV)-associated diseases. It resembles hydroa vacciniforme (HV), the rarest form of photosensitivity, and is a T-cell disorder associated with an Epstein-Barr virus infection. The majority of diagnosed cases occur in East Asia and South America. It is rare in the United States and Europe. Multiple studies have revealed the clinical manifestation of an enlarged liver, but no gold standard such as pathology has yet supported this as a clinical sign of HV-LPD.

CASE PRESENTATION

Here, we report a case of a 34-year-old Asian female with definite liver invasion. The patient had complained of a recurring facial rash for many years. The patient was admitted to the hospital because of an enlarged liver. After hospitalization, she was given an EB virus nucleic acid test. The EB virus nucleic acid test was positive, and pathological examination suggested that HV-LPD had invaded the skin, bone marrow, and liver. After being given antiviral treatment, the patient's symptoms were mitigated.

CONCLUSIONS

Our case confirms the liver damage was caused by HV-LPD and the effectiveness of antiviral treatment.

摘要

背景

类水疱样疹样淋巴组织增生性疾病(HV-LPD)是一组与 EBV 相关的疾病的名称。它类似于最罕见的光敏性疾病水疱样疹(HV),是一种与 EBV 感染相关的 T 细胞疾病。大多数诊断病例发生在东亚和南美洲。它在美国和欧洲很少见。多项研究揭示了肝脏肿大的临床表现,但尚无病理学等金标准支持这是 HV-LPD 的临床标志。

病例介绍

这里,我们报告了一例 34 岁亚洲女性,存在明确的肝脏侵犯。该患者多年来反复出现面部皮疹。因肝脏肿大,患者入院治疗。住院后,进行了 EBV 核酸检测。EBV 核酸检测阳性,病理检查提示 HV-LPD 侵犯皮肤、骨髓和肝脏。给予抗病毒治疗后,患者症状缓解。

结论

我们的病例证实了肝脏损伤是由 HV-LPD 引起的,以及抗病毒治疗的有效性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c549/7788891/a3037e9d0414/12879_2020_5697_Fig1_HTML.jpg

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