ArticleOrphanet journal of rare diseases2023
Sintilimab treatment for chronic active Epstein-Barr virus infection and Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in children.
Article in Orphanet journal of rare diseases, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.
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11 citing papers in PubMed, 17 citations in OpenAlex.
- Review
- Exploratory study of PD-1 inhibitors with or without ruxolitinib for the treatment of adult EBV-associated hemophagocytic lymphohistiocytosis: a real-world data-based preliminary investigation.Clinical and experimental medicine · 2026Article
- Comparison of the efficacy of transarterial chemoembolization combined with radiofrequency ablation or sintilimab and bevacizumab for unresectable hepatocellular carcinoma.BMC gastroenterology · 2026Article
- Splenectomy does not improve survival in chronic active Epstein-Barr virus disease patients.Annals of hematology · 2025Article
- Pilot Study of Anti-PD-1 Antibody Combined with L-DEP Regimens in the Treatment of Relapsed/Refractory EBV-HLH in Children.Journal of clinical immunology · 2025Article
- Treating and triggering hyperinflammation: tackling hemophagocytic lymphohistiocytosis and HLH-like syndromes in the pediatric cell therapy and critical care setting.Frontiers in oncology · 2025Review
- Hemophagocytic lymphohistiocytosis: current treatment advances, emerging targeted therapy and underlying mechanisms.Journal of hematology & oncology · 2024Review
- PD-1 blockade immunotherapy as a successful rescue treatment for disseminated adenovirus infection after allogeneic hematopoietic stem cell transplantation.Journal of hematology & oncology · 2024Article
- Case report: emapalumab treatment for a pediatric Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) patient with cytokine storm enabling allogeneic hematopoietic cell transplantation.Translational pediatrics · 2024Article
- PD-1 immunology in the kidneys: a growing relationship.Frontiers in immunology · 2024Review
- Role of rapidly evolving immunotherapy in chronic active Epstein-Barr virus disease.Frontiers in immunology · 2024Review
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Authors and funding
11 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundChronic active Epstein-Barr virus infection (CAEBV) and Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) are rare but life-threatening progressive diseases triggered by EBV infection. Glucocorticoid/immunosuppressants treatment is temporarily effective; however, most patients relapse and/or progress. Hematopoietic stem cell transplantation (HSCT) is a potentially curative therapy; however, there are risks of transplantation-associated complications. Currently there is no standard treatment for CAEBV and EBV-HLH. Programmed death protein 1 (PD-1) inhibitors have achieved a high response in many EBV-related diseases. Sintilimab (a recombinant human IgG4 monoclonal antibody against PD-1) disrupts the interaction between PD-1 and its ligand, leading to T cell reinvigoration.
methodsA retrospective analysis was performed on three children with CAEBV or EBV-HLH in the Children's Hospital of Soochow University between 12 December 2020 and 28 November 2022. The efficacy of sintilimab was evaluated.
resultsThree patients, including two males and one female, were analyzed. Among them, two children were diagnosed with CAEBV with intermittent fever for more than four years, and one child was diagnosed with EBV-HLH. After sintilimab treatment and a mean follow-up of 17.1 months (range 10.0-23.3 months), patients 1 and 3 achieved a complete clinical response and patient 2 achieved a partial clinical response. All three children showed a > 50% decrease in EBV-DNA load in both blood and plasma. EBV-DNA copies in sorted T, B, and NK cells were also markedly decreased after sintilimab treatment.
conclusionOur data supported the efficacy of PD-1 targeted therapy in certain patients with CAEBV and EBV-HLH, and suggested that sintilimab could provide a cure for these diseases, without HSCT. More prospective studies and longer follow-up are needed to confirm these conclusions.
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