ORPHA:364033
Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood
Also known as: Systemic EBV+ T-cell LPD of childhood · Systemic EBV-positive T-cell lymphoproliferative disease of childhood
Publications
129
66.2th percentile
Trials
0
Interventional, condition-specific
Researchers
801
Distinct authors in sample
Gene link
—
Readiness
1/6
Stages with a signal
Clinical definition (Orphanet)
A rare and very aggressive neoplastic disease emerging after a primary acute or chronic active EBV infection. It presents with persisting fever and malaise, 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 .
How rare: How common this is has not been clearly measured.
Cross-references
Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.
- MONDO:0018223
- UMLS:C2699747
- NCIT:C80374
Additional Mondo synonyms (4)
systemic EBV+ T-cell LPD of childhood · systemic EBV-positive T-cell lymphoma of childhood · systemic EBV-positive T-cell lymphoproliferative disease of childhood · systemic EBV-positive T-cell lymphoproliferative disorder of childhood
Research stages
Trial readiness signals
Where this condition sits on an open-data research pipeline — not how close a treatment is, and not medical advice. Empty stages often mean “not in these databases under this Mondo ID,” not “impossible.”
1/6 stages with a signal
Research-stage checklist from open sources (GenCC, literature, Monarch when enriched, ClinicalTrials.gov). Not a prognosis or care recommendation.
- Gene identifiedNot found
No GenCC disease–gene assertion in this build
- LiteraturePresent
129 matched papers (99 in last 10 years) Source
- Phenotype characterisedNot checked
Not yet enriched from Monarch / HPO
- Animal modelNot checked
Not yet enriched from Monarch / Alliance
- Orphan designationNot checked
FDA/EMA orphan-drug designation not enriched yet
- Interventional trialNot found
No matched interventional trial under our ClinicalTrials.gov rules
Biology
Genes and phenotypes
Gene–disease validity from GenCC, plus phenotypes and animal models joined from Monarch Initiative via Mondo ID — not a clinical diagnosis aid.
Do we know what causes it?
Not yet — the cause hasn't been pinned down in GenCC.
No strong gene–disease assertion joined for this Orphanet entity.
Phenotypes (Monarch / HPO)
Not enriched in this build — Monarch phenotype joins were not run for this record.
Animal models (Monarch / Alliance)
Not enriched in this build.
Literature
Is anyone studying this?
129
129 papers have ever been published on this condition (under this name). For scale, breast cancer has over 700,000. Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=5449) is 41.
129 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 41 (publications denominator n=5449).
99 in the last 10 years · high confidence · 66.2th percentile (publications denominator)
Phrase hits: 129 · MeSH hits: 0
Who's working on it?
801
Distinct author names in 129 sampled papers — named people below.
Who's working on it?
No interventional trial matched this name; these authors publish on it in the sampled literature — a practical starting point for contact.
- 01Jaffe ES10 papers · 2023
Laboratory of Pathology, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA. Electronic address: elainejaffe@nih.gov.
Papers in Europe PMC - 02Quintanilla-Martinez L10 papers · 2025
Institute of Pathology and Neuropathology, Eberhard Karls University of Tübingen and Comprehensive Cancer Center, University Hospital Tübingen, Tübingen, Germany.
Papers in Europe PMC - 03Kimura H9 papers · 2026
Department of Virology, Nagoya University Graduate School of Medicine, Nagoya.
Papers in Europe PMC - 04Fend F6 papers · 2026
Institute of Pathology and Comprehensive Cancer Center, Tübingen University Hospital, Tübingen, Germany.
Papers in Europe PMC - 05Chen Z5 papers · 2026
Key Laboratory of Artificial Organs and Computational Medicine of Zhejiang Province, Shulan International Medical College, Zhejiang Shuren University, 8 Shuren St, Gongshu District, Hangzhou, 310015, Zhejiang Province, People's Republic of China.
Papers in Europe PMC - 06Fujiwara S5 papers · 2020
Department of Allergy and Clinical Immunology, National Research Institute for Child Health and Development, Tokyo 157-8535, Japan.
Papers in Europe PMC - 07Ng SB5 papers · 2023
Cancer Science Institute of Singapore, National University of Singapore, Singapore, Singapore.
Papers in Europe PMC - 08Pittaluga S5 papers · 2021
Hematopathology Section, Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, National Institutes of Health (NIH), Bethesda, Maryland, USA.
Papers in Europe PMC - 09Wang M5 papers · 2026
Department of Dermatology and Venerology, West China Hospital of Sichuan University , Chengdu , China.
Papers in Europe PMC - 10Zhao S5 papers · 2026
Division of Gastroenterology and Hepatology, Key Laboratory of Gastroenterology and Hepatology, Ministry of Health, Shanghai Institute of Digestive Disease, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China.
Papers in Europe PMC
Clinical research
Is a treatment being tested?
0
interventional trials for this specific condition
No interventional trial testing a treatment matched this specific condition name. 1 observational study did — shown below because natural-history and cohort work can be an important step toward a trial.
Data as of 27 July 2026
No matched interventional trials. This is true for 73% of diseases in the trials denominator (5114 of 7003). Here are the researchers publishing on it.
high confidence · 36.5th percentile (trials denominator)
Recruiting interventional trials
From the matched ClinicalTrials.gov set
No interventional trial testing a treatment was found for this specific condition name on ClinicalTrials.gov.
See who's working on it — people publishing on this disease are often the practical next contact when no trial is listed.
Observational and natural-history studies
1 observational study matches this condition. These do not test a treatment and are not counted in the interventional-trial headline, but they are genuine research: natural-history work often defines the endpoints needed for a future rare-disease trial, and families may be able to enroll.
Recruiting or not-yet-recruiting
- NCT05978141·RECRUITING·A Registry for People With T-cell Lymphoma
Conditions: T-cell Lymphoma · NK-Cell Lymphoma · T-cell Prolymphocytic Leukemia · T-cell Large Granular Lymphocytic Leukemia·Matched via name phrase
Where to find support
We do not yet link condition-specific patient organisations. These umbrella groups support undiagnosed and ultra-rare families:
India — NPRD
Last verified 2026-07-26This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.
Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.
How we counted this
Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored on the overview but are not added to the query string.
"Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood" OR "Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of the childhood" OR "Systemic EBV+ T-cell LPD of childhood" OR "Systemic EBV+ T-cell LPD of the childhood" OR "Systemic EBV-positive T-cell lymphoproliferative disease of childhood" OR "Systemic EBV-positive T-cell lymphoproliferative disease of the childhood" OR "systemic EBV-positive T-cell lymphoma of childhood" OR "systemic EBV-positive T-cell lymphoma of the childhood" OR "systemic EBV-positive T-cell lymphoproliferative disorder of childhood" OR "systemic EBV-positive T-cell lymphoproliferative disorder of the childhood"
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood" OR "Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of the childhood" OR "Systemic EBV+ T-cell LPD of childhood" OR "Systemic EBV+ T-cell LPD of the childhood" OR "Systemic EBV-positive T-cell lymphoproliferative disease of childhood" OR "Systemic EBV-positive T-cell lymphoproliferative disease of the childhood" OR "systemic EBV-positive T-cell lymphoma of childhood" OR "systemic EBV-positive T-cell lymphoma of the childhood" OR "systemic EBV-positive T-cell lymphoproliferative disorder of childhood" OR "systemic EBV-positive T-cell lymphoproliferative disorder of the childhood"
Study-type breakdown: 0 interventional · 1 observational · 0 expanded access. Only interventional studies enter the trial headline.
Query health: ok — strategies attempted: phrase; with hits: phrase
Run this search on ClinicalTrials.gov
Confidence reasoning
- Preferred label is multi-word and distinctive
- No synonyms dropped by stoplist
- No label/synonym collisions with other diseases in this corpus
Ingested 2026-07-27T14:46:27.034Z