Massive pericardial effusion due to chronic active Epstein-Barr virus infection successfully treated with PD-1 blockade: A case report

Medicine (Baltimore). 2022 Aug 26;101(34):e30298. doi: 10.1097/MD.0000000000030298.


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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Chronic Disease
  • Epstein-Barr Virus Infections* / drug therapy
  • Epstein-Barr Virus Infections* / therapy
  • Female
  • Herpesvirus 4, Human / genetics
  • Humans
  • Lymphoproliferative Disorders* / complications
  • Lymphoproliferative Disorders* / drug therapy
  • Pericardial Effusion* / etiology
  • Pericardial Effusion* / therapy
  • Persistent Infection
  • Programmed Cell Death 1 Receptor / therapeutic use


  • Programmed Cell Death 1 Receptor