Fatal refractory chronic active Epstein-Barr virus infection with hemophagocytic lymphohistiocytosis and NK/T-cell lymphoma: a case report.

ASM case reports Pub Date : 2026-02-09 eCollection Date: 2026-03-01 DOI:10.1128/asmcr.00168-25

Nicholas Mielke, Marlaena Nooney, Nagendra Natarajan, Rima El-Herte

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Abstract

Background: Epstein-Barr virus (EBV) is most often associated with asymptomatic infection or infectious mononucleosis syndrome. A rare and fatal underestimated complication is chronic active EBV infection (CAEBV) with hemophagocytic lymphohistiocytosis (HLH) and B or NK/T-cell lymphoma.

Case summary: A 28-year-old woman presented with fevers, malaise, fatigue, abdominal pain, nausea, vomiting, and episodic skin rashes. Laboratory tests revealed pancytopenia, significantly elevated liver enzymes, and serology consistent with primary EBV infection, with very high EBV DNA in serum. All other causes of hepatitis were excluded. Liver biopsy showed EBV-positive T-cells infiltrating the liver parenchyma with erythrophagocytosis, consistent with CAEBV infection with HLH. Despite aggressive interventions, the patient ultimately succumbed to grave complications and refractory disease.

Conclusion: CAEBV is a rare and fatal complication of EBV. Clinician education, early recognition, and expert consultation improve outcomes. Treatment so far consists of chemotherapy followed by hematopoietic stem cell transplantation.

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致死性难治性慢性活动性eb病毒感染合并噬血细胞淋巴组织细胞增多症和NK/ t细胞淋巴瘤1例

背景：eb病毒（EBV）最常与无症状感染或传染性单核细胞增多症相关。慢性活动性EBV感染（CAEBV）伴噬血细胞性淋巴组织细胞增多症（HLH）和B或NK/ t细胞淋巴瘤是一种罕见且致命的低估并发症。病例总结：一名28岁女性，表现为发热、不适、疲劳、腹痛、恶心、呕吐和间歇性皮疹。实验室检查显示全血细胞减少，肝酶显著升高，血清学与原发性EBV感染一致，血清中EBV DNA含量非常高。排除了所有其他肝炎原因。肝活检显示ebv阳性t细胞浸润肝实质伴红细胞吞噬，与CAEBV感染合并HLH一致。尽管采取了积极的干预措施，患者最终还是死于严重的并发症和难治性疾病。结论：CAEBV是一种罕见且致命的EBV并发症。临床医生教育、早期识别和专家咨询可改善预后。目前的治疗包括化疗和造血干细胞移植。

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ASM case reports

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