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A rare and very aggressive neoplastic disease emerging after a primary acute or chronic active EBV infection. It presents with persisting fever and malaise, hepatosplenomegaly with or without lymphadenopathy, liver failure, severe pancytopenia and a rapid progression towards multi-organ failure and hemophagocytic syndrome with a fatal issue. It is characterized by clonal proliferation of EBV-infected T cells with an activated cytotoxic phenotype.
Biomarker and diagnostic research for systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood has been reported in the published literature.
Estimated prevalence: Unknown (Unknown prevalence).
1 clinical trial registered, 1 recruiting. Interventions under study include other interventions. Research is primarily sponsored by academic and government institutions.
11 publications have been identified in PubMed for systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood. Research spans Case Report / Case Series (55%), Review / Meta-Analysis (27%), and Diagnostic / Biomarker (9%).
Research Type | Count | % of Total |
|---|---|---|
Patient case studies | 6 |
Data assembled from 4 of 12 sources · Last updated Sep 21, 2026, 4:55 AM UTC
European rare disease database
Genetic and Rare Diseases Info Center
Research summaries | 3 | 27% |
Testing and diagnosis research | 1 | 9% |
Disease patterns and progression | 1 | 9% |
Anya OO (2026). [PMID: 42261517](https://pubmed.ncbi.nlm.nih.gov/42261517/). *Cureus*. [Case Report / Case Series]
Luniewski A (2026). [PMID: 41657941](https://pubmed.ncbi.nlm.nih.gov/41657941/). *Lymphatics*. [Review / Meta-Analysis]
Guan P (2026). [PMID: 41729084](https://pubmed.ncbi.nlm.nih.gov/41729084/). *JCI insight*. [Diagnostic / Biomarker]
García-González S (2025). [PMID: 39938780](https://pubmed.ncbi.nlm.nih.gov/39938780/). *Actas dermo-sifiliograficas*. [Review / Meta-Analysis]
Ma L (2025). [PMID: 41306545](https://pubmed.ncbi.nlm.nih.gov/41306545/). *Frontiers in pediatrics*. [Case Report / Case Series]
Coman D (2025). [PMID: 41725795](https://pubmed.ncbi.nlm.nih.gov/41725795/). *Canadian liver journal*. [Case Report / Case Series]
Lerner L (2024). [PMID: 39493602](https://pubmed.ncbi.nlm.nih.gov/39493602/). *Journal of hematology*. [Case Report / Case Series]
Dilmurat D (2024). [PMID: 38956696](https://pubmed.ncbi.nlm.nih.gov/38956696/). *Italian journal of pediatrics*. [Epidemiology / Natural History]
Jimenez A (2024). [PMID: 39185704](https://pubmed.ncbi.nlm.nih.gov/39185704/). *Pediatric blood & cancer*. [Case Report / Case Series]
Cao P (2024). [PMID: 39196630](https://pubmed.ncbi.nlm.nih.gov/39196630/). *Journal of pediatric hematology/oncology*. [Review / Meta-Analysis]
AI-curated news mentioning systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood
Updated Aug 25, 2026
A recent study highlights the acute development of Epstein-Barr virus-positive T-cell lymphoproliferative disorder following rituximab treatment for follicular lymphoma. This finding underscores the need for vigilance in monitoring patients receiving rituximab.
A pediatric case report highlights a rare instance of Epstein-Barr virus-positive B-cell lymphoproliferative disorder complicated by septic shock in a patient with activated PI3Kδ syndrome. This study contributes to the understanding of the clinical implications of these conditions.
A recent study highlights the cytological features of Epstein-Barr virus-positive nodal T- and NK-cell lymphoma, a rare and aggressive form of lymphoma. This research contributes to the understanding of the disease's characteristics, which may aid in diagnosis and treatment.
FDA issues a second complete response letter to Atara Biotherapeutics for its T-cell immunotherapy tabelecleucel (Ebvallo) aimed at treating EBV-positive posttransplant lymphoproliferative disease. This decision raises concerns about barriers to clinical evidence generation for ultra-rare conditions, potentially delaying patient access to critical therapies.