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Updated: May 26, 2026
An Efficient and Simple Method to Establish NK and T Cell Lines from Patients with Chronic Active Epstein-Barr Virus Infection
Published on: March 30, 2018
Epstein-Barr virus-positive systemic NK/T-cell lymphomas in children: report of six cases
Socorro M Rodríguez-Pinilla1, Carlos Barrionuevo, Juan García
1Lymphoma Group, Molecular Pathology Programme, Spanish National Cancer Research Centre (CNIO), Madrid, Spain. smrodriguez@cnio.es
Insights
This study details six pediatric Epstein-Barr virus (EBV)-positive T-cell lymphomas with aggressive progression and poor survival. These findings suggest a distinct form of EBV-positive T-cell lymphoproliferative disease requiring targeted therapies.
Area of Science:
- Pediatric Hematology Oncology
- Immunology
- Virology
Background:
- The World Health Organization classifies Epstein-Barr virus (EBV)-positive T-cell lymphoproliferative disorders in children into two types.
- Systemic EBV-positive T-cell lymphoproliferative disease of childhood and hydroa vacciniforme-like lymphoma are recognized entities.
- Hydroa vacciniforme-like lymphoma is more common in Asia and Latin America.
Observation:
- Six male pediatric patients (median age 9 years) presented with acute symptoms including fever, weight loss, and enlarged liver/spleen.
- Affected sites included lymph nodes, gut, lungs, and abdominal wall soft tissues.
- Histopathology revealed lymph node replacement by pleomorphic atypical cells, vasculitis, and necrosis.
Findings:
- Neoplastic cells expressed EBV-encoded RNA, T-cell markers (CD2, CD3), and cytotoxic markers (TIA1, granzyme-B, perforin).
- CD56 and T-cell receptor-gamma were positive in one case each; TCR-BF1, CD4, CD8, and ALK were negative.
- All patients experienced rapid disease progression, with a median survival of 7.1 months.
Implications:
- These cases likely represent a distinct solid form of systemic EBV-positive T-cell lymphoproliferative disease of childhood.
- Accurate identification of this specific T-cell lymphoma subtype is crucial.
- Further research is needed to develop effective therapeutic strategies for this aggressive pediatric malignancy.
Aims:
The World Health Organization lymphoma classification recognizes two different Epstein-Barr virus (EBV)-positive T-cell lymphoproliferative disorders of childhood: systemic EBV-positive T-cell lymphoproliferative disease of childhood, and hydroa vacciniforme-like lymphoma, which is more prevalent in Asia and Latin America. The aim of this study was to characterize six cases of paediatric EBV-positive peripheral T-cell lymphoma with distinct features.
Methods And Results:
All cases were male, with a median patient age of 9 years (range: 5-17 years). Most of them presented suddenly with fever, weight loss, hepatosplenomegaly, peripheral lymphadenopathy, and high lactate dehydrogenase (LDH) levels. Moreover, gut, lung or soft tissues of the abdominal wall were also affected in four cases. Partial to total replacement of the lymph node by pleomorphic infiltration of atypical neoplastic cells was found in all cases. Vasculitis and geographical areas of necrosis were seen in three and four cases, respectively. Neoplastic cells showed expression of EBV-encoded RNA, T-cell markers (CD2 and CD3), and cytotoxic markers (TIA1, granzyme-B, and perforin). CD56 and T-cell receptor -γ were expressed in one case each. TCR-BF1, CD4, CD8 and anaplastic lymphoma kinase were negative. In all cases, the disease progressed rapidly, causing death of the patient, with a median survival of 7.1 months (range: 1-13 months).
Conclusions:
These cases probably represent a solid form of systemic EBV-positive T-cell lymphoproliferative disease of childhood, which requires identification and the development of appropriate therapy.
