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Massive pericardial effusion due to chronic active Epstein-Barr virus infection successfully treated with PD-1
Yubo Pi1, Jingshi Wang1, Zhao Wang1
1Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing, China.
Rationale:
Chronic 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 Concerns:
A 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.
Diagnosis:
High 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.
Interventions:
The patient received sintilimab injection at a dose of 200 mg every 2 weeks in combined with lenalidomide 10 mg once daily.
Outcomes:
The patient achieved complete resolution of pericardial effusion 5 months after PD-1 blockade immunotherapy without apparent adverse effects.
Lessons:
CAEBV 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.
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