EBV-positive T/NK cell lymphoproliferative disease (EBV-T/NK-LPD) in children is a rare, highly aggressive, and potentially fatal heterogeneous disease, encompassing subtypes such as chronic active EBV infection (CAEBV), pediatric systemic EBV-positive T-cell lymphoma, and vaccinia vesicular disease-like lymphoproliferative disease. It is prone to progress to hemophagocytic lymphohistiocytosis (HL
Conditions
Interventions
Observation group (including chronic active EBV infection (CAEBV), systemic EBV-positive T-cell lymphoma in children, severe mosquito bites, and vaccine-like lymphoproliferative disorders):None
Sponsors
Beijing Childrens Hospital,Capital Medical University
Eligibility
Sex/Gender
All
Age
No minimum to 18 Years
Inclusion criteria
Inclusion criteria: 1. Age <= 18 years old, gender is not limited; 2. Diagnosed as EBV-positive T/NK cell lymphoproliferative disease, in accordance with the 2022 WHO classification of lymphoid neoplasms; 3. Receive allo-HSCT (identical sibling, unrelated, haploid, cord blood transplantation) at this center; 4. Clinical data, transplantation data, and follow-up records are complete.
Exclusion criteria
Exclusion criteria: 1. EBV-negative or B-cell-derived lymphoproliferative disease; 2. Autologous hematopoietic stem cell transplantation, second transplantation/multiple transplantation; 3. Combined with congenital immune deficiency disease, primary HLH, and other malignant tumors; 4. Serious missing clinical data, loss to follow-up, and abandonment of treatment midway.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Overall Survival (OS);Event-Free Survival (EFS);2-year, 3-year, and 5-year OS and EFS rates; | — |
Secondary
| Measure | Time frame |
|---|---|
| Cumulative Incidence of Relapse (CIR);Transplant-Related Mortality (TRM);Time to Neutrophil and Platelet Engraftment;Incidence and Severity of Acute and Chronic Graft-Versus-Host Disease (GVHD);Reactivation Rate, Peak Level, and Control Rate of EBV-DNA After Transplantation;Incidence of Severe Infections (Bacterial, Fungal, Viral);Incidence of Organ Toxicity and Complications; | — |
Countries
China
Contacts
Public ContactYang Jun
Beijing Childrens Hospital,Capital Medical University
Outcome results
None listed