• Yubo Pi, Jingshi Wang, and Zhao Wang.
• Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing, China.
• Medicine (Baltimore). 2022 Aug 26; 101 (34): e30298.

RationaleChronic active Epstein-Barr virus (EBV) infection (CAEBV) is a rare but life-threatening EBV-positive lymphoproliferative disorder. Currently, treatment options for CAEBV are limited. Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is the only way to cure CAEBV. Here, we report a rare case of CAEBV manifesting as massive pericardial effusion that was successfully treated with programmed cell death protein-1 (PD-1) blockade immunotherapy.Patient ConcernsA 36-year-old woman with intermittent chest distress and dyspnea for 8 months was admitted to our center on October 25, 2021. Laboratory tests showed leukocytopenia and elevated liver enzyme levels. Initial echocardiography revealed massive pericardial effusion.DiagnosisHigh levels of EBV-DNA were detected in the pericardial fluid by metagenomic next-generation sequencing. The pathological diagnosis of her left inguinal lymph node and skin lesions revealed systemic CAEBV.InterventionsThe patient received sintilimab injection at a dose of 200 mg every 2 weeks in combined with lenalidomide 10 mg once daily.OutcomesThe patient achieved complete resolution of pericardial effusion 5 months after PD-1 blockade immunotherapy without apparent adverse effects.LessonsCAEBV is a rare but life-threatening EBV-positive lymphoproliferative disease. We present a rare case of massive pericardial effusion caused by systemic CAEBV, which was successfully treated with sintilimab. This case highlights the promising curative effect of PD-1 blockade immunotherapy in systemic CAEBV, especially for patients not suitable for allo-HSCT.Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.

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