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Updated: May 27, 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
EBV-associated T/NK-cell lymphoproliferative diseases in nonimmunocompromised hosts: prospective analysis of 108
Hiroshi Kimura1, Yoshinori Ito, Shinji Kawabe
1Departments of Virology, Nagoya University Graduate School of Medicine, Japan. hkimura@med.nagoya-u.ac.jp
Insights
Epstein-Barr virus-associated T/NK-cell lymphoproliferative disease (EBV-T/NK-LPD) involves clonal T or NK cell growth. Key risk factors for mortality include older age at onset and liver dysfunction, with transplantation improving outcomes.
Area of Science:
- Hematology
- Immunology
- Oncology
Background:
- Epstein-Barr virus-associated T/NK-cell lymphoproliferative disease (EBV-T/NK-LPD) is a severe systemic illness.
- It is characterized by the clonal expansion of Epstein-Barr virus (EBV)-infected T or NK cells.
- Understanding its clinical spectrum and outcomes is crucial for patient management.
Purpose of the Study:
- To prospectively characterize the clinical features, immunophenotypes, and outcomes of nonimmunocompromised patients with EBV-T/NK-LPD.
- To identify risk factors for mortality and prognostic indicators.
- To provide insights into diagnostic and therapeutic strategies for distinct EBV-T/NK-LPD subtypes.
Main Methods:
- Prospective enrollment of 108 nonimmunocompromised patients with EBV-T/NK-LPD.
- Clinical categorization into four groups: chronic active EBV disease, EBV-associated hemophagocytic lymphohistiocytosis, severe mosquito bite allergy, and hydroa vacciniforme.
- Median follow-up of 46 months, assessing mortality, development of lymphoma/leukemia, and treatment outcomes including hematopoietic stem cell transplantation.
Main Results:
- Of 108 patients, 44% died from organ complications; 13 developed overt lymphoma/leukemia.
- Clinical profiles correlated with EBV(+) T-cell (64 cases) or NK-cell (44 cases) immunophenotypes.
- Age at onset (≥ 8 years) and liver dysfunction were mortality risk factors; hematopoietic stem cell transplantation (59 patients) resulted in a 66% survival rate.
Conclusions:
- Systemic EBV-T/NK-LPD has diverse clinical presentations linked to specific cell immunophenotypes.
- Early identification of risk factors like age and liver dysfunction is critical for prognosis.
- Hematopoietic stem cell transplantation offers a survival benefit, guiding therapeutic approaches for this challenging disease.
Abstract:
EBV-associated T/NK-cell lymphoproliferative disease (T/NK-LPD) is defined as a systemic illness characterized by clonal proliferation of EBV-infected T or NK cells. We prospectively enrolled 108 nonimmunocompromised patients with this disease (50 men and 58 women; median onset age, 8 years; age range, 1-50 years) evidenced by expansion of EBV(+) T/NK cells in the peripheral blood; these were of the T-cell type in 64 cases and of the NK-cell type in 44, and were clinically categorized into 4 groups: 80 cases of chronic active EBV disease, 15 of EBV-associated hemophagocytic lymphohistiocytosis, 9 of severe mosquito bite allergy, and 4 of hydroa vacciniforme. These clinical profiles were closely linked with the EBV(+) cell immunophenotypes. In a median follow-up period of 46 months, 47 patients (44%) died of severe organ complications. During the follow-up, 13 patients developed overt lymphoma or leukemia characterized by extranodal NK/T-cell lymphoma and aggressive NK-cell leukemia. Fifty-nine received hematopoietic stem cell transplantation, 66% of whom survived. Age at onset of disease (≥ 8 years) and liver dysfunction were risk factors for mortality, whereas patients who received transplantation had a better prognosis. These data depict clinical characteristics of systemic EBV(+) T/NK-LPD and provide insight into the diagnostic and therapeutic approaches for distinct disease.
