关键词: Acute infection EB virus NK/T-cell lymphoma T/natural killer cells

Mesh : Humans Infectious Mononucleosis / immunology virology diagnosis Male Female Child Adolescent Herpesvirus 4, Human / immunology Killer Cells, Natural / immunology Epstein-Barr Virus Infections / immunology virology Retrospective Studies Child, Preschool Adult Young Adult Lymph Nodes / pathology immunology virology T-Lymphocytes / immunology

来  源:   DOI:10.1007/s12308-024-00595-6   PDF(Pubmed)

Abstract:
Epstein-Barr virus (EBV) typically infects B cells in infectious mononucleosis (IM), but a rare case shows EBV infection in T cells. Seven cases of lymphoproliferative disorder caused by EBV-positive cytotoxic T/natural killer (NK) cell proliferation in the lymph nodes, termed IM with transient EBV infection of T and NK cells (EBV + T/NK cells in IM), are reported here. The purpose of the study is to describe clinicopathological features of EBV + T/natural killer (NK) cells in IM of the lymph node. We retrospectively analysed seven cases of Chinese children and young people adults with EBV + T/NK cells in IM. We used morphological observation, immunohistochemical staining, EB virus in situ hybridisation detection, and analysis of T-cell receptor gene rearrangement. The patients were healthy prior to illness, experiencing sudden onset occurring in all the patients, with high fever as the first symptom, followed by lymphadenopathy and hepatosplenomegaly. Diagnosis occurred < 1.5 months of symptom onset. Most lymphocytes in lesions expressed CD3 and Granzyme B or TIA-1 and lacked CD5. CD56 was expressed in numerous cells in 5 of the 7 cases. EBV-encoded RNA (EBER) was detected in medium-to-large-sized cells (50-100 cells per cell/high-power field). T-cell receptor (TCR) gene rearrangement was seen in six cases, with monoclonal rearrangement in four cases. Treatment was conservative treatment but not chemotherapy. Four received anti-HLH therapy and others anti-inflammatory treatment. All patients survived with relapse after long-term clinical observation and follow-up. EBV + T/NK cells in IM can elicit malignant features that mimic T/NK-cell lymphoma pathologically and benign features mimicking IM clinically. These findings indicate that EBV + T/NK cells in IM could serve as valuable diagnosis. Additional clinical information, including age of onset (children and young people), nature of onset (sudden), disease course (short), symptoms (systemic), EBV infection status (acute), and lymph node involvement, is crucial for accurate diagnosis and prognostic evaluation.
摘要:
EB病毒(EBV)通常会感染传染性单核细胞增多症(IM)中的B细胞,但一个罕见的病例显示EBV感染T细胞。淋巴结EBV阳性细胞毒性T/自然杀伤(NK)细胞增殖引起的淋巴增生性疾病7例,称为IM,伴有T和NK细胞的短暂EBV感染(IM中的EBV+T/NK细胞),在这里报道。该研究的目的是描述淋巴结IM中EBVT/自然杀伤(NK)细胞的临床病理特征。我们回顾性分析了7例IM中EBVT/NK细胞的中国儿童和年轻人。我们用形态学观察,免疫组织化学染色,EB病毒原位杂交检测,T细胞受体基因重排分析。病人在生病前是健康的,所有患者都经历突然发作,以高烧为首发症状,其次是淋巴结肿大和肝脾肿大。诊断发生在症状发作<1.5个月。病变中的大多数淋巴细胞表达CD3和颗粒酶B或TIA-1,缺乏CD5。7例中有5例CD56在众多细胞中表达。在中型至大型细胞(每个细胞50-100个细胞/高倍视野)中检测到EBV编码的RNA(EBER)。6例T细胞受体(TCR)基因重排,4例单克隆重排。治疗为保守治疗,不进行化疗。4人接受抗HLH治疗和其他抗炎治疗。经过长期的临床观察和随访,所有患者均存活,复发。IM中的EBV+T/NK细胞可引起病理上模拟T/NK细胞淋巴瘤的恶性特征和临床上模拟IM的良性特征。这些发现表明IM中的EBVT/NK细胞可以作为有价值的诊断。其他临床信息,包括发病年龄(儿童和年轻人),发病性质(突然),病程(短),症状(全身),EBV感染状态(急性),淋巴结受累,对于准确诊断和预后评估至关重要。
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