Epstein–Barr virus‐associated T/natural killer‐cell lymphoproliferative disorders

  title={Epstein–Barr virus‐associated T/natural killer‐cell lymphoproliferative disorders},
  author={Sanghui Park and Young Hyeh Ko},
  journal={The Journal of Dermatology},
  • Sanghui Park, Y. Ko
  • Published 1 January 2014
  • Medicine, Biology
  • The Journal of Dermatology
Primary infection with Epstein–Barr virus (EBV) is usually asymptomatic and, in a normal host, EBV remains latent in B cells after primary infection for the remainder of life. Uncommonly, EBV can infect T or natural killer (NK) cells in a person with a defect in innate immunity, and EBV infection can cause unique systemic lymphoproliferative diseases (LPD) of childhood. Primary infection in young children can be complicated by hemophagocytic lymphohistiocytosis or fulminant systemic T‐cell LPD… 
Epstein-Barr Virus-Associated T and NK-Cell Lymphoproliferative Diseases
The EBV-associated hemophagocytic lymphohistiocytosis (HLH), although not included in the WHO classification because it is a reactive, inflammatory disease, is included in this review because it can be life-threatening and may have overlapping features with other EBV+ T/NK LPDs.
Epstein-Barr Virus Type 2 Latently Infects T Cells, Inducing an Atypical Activation Characterized by Expression of Lymphotactic Cytokines
The novel finding that EBV-2 infects T cells in culture will provide a model to understand the role EBV plays in the development of T-cell lymphomas and lay the foundation for further studies to elucidate the role of EBV in the pathogenesis of T -cell lymphoproliferative diseases.
Epstein-Barr Virus–Associated Lymphoproliferative Disorders: Review and Update on 2016 WHO Classification
This article reviews the current evidence covering EBV-associated LPDs based on the 2016 classification of the World Health Organization and explains why it is important to understand their unique pathophysiology for correct diagnoses and optimal management.
EBV-positive T/NK lymphoproliferative diseases: analysis of prognostic factors for patients in China.
It was observed that the high mortality rate of EBV + T/NK-LPD was mainly due to serious and fatal complications, and disease prognosis should be based on a comprehensive analysis of pathological and clinical data.
Chronic active EBV infection associated with NK cell lymphoma and hemophagocytic lymphohistiocytosis in a 27-year-old woman
In the clinical setting of unexplained fever and liver injury, it is necessary to be aware of CAEBV, as well as its fatal complication such as EBV associated NK cell lymphoma and HLH.
EBV-Positive Lymphoproliferations of B- T- and NK-Cell Derivation in Non-Immunocompromised Hosts
The definitions, morphology, pathogenesis, and evolving concepts of the various EBV-associated disorders including EBV+ diffuse large B-cell lymphoma, not otherwise specified,EBV+ mucocutaneous ulcer, DLBCL associated with chronic inflammation, fibrin-associatedDLBCL, lymphomatoid granulomatosis are reviewed.
Gene expression analysis of hypersensitivity to mosquito bite, chronic active EBV infection and NK/T-lymphoma/leukemia
Unsupervised hierarchal clustering analyses of microarray data and gene ontology analysis revealed specific gene clusters and identified several candidate genes responsible for disease that can be used to discriminate each category of NK-LPD and NK-cell lymphoma/leukemia.
Comprehensive assessment of peripheral blood TCRβ repertoire in infectious mononucleosis and chronic active EBV infection patients
This study fully characterized the diversity of peripheral blood TCRβ repertoire in IM and CAEBV patients and observed some disease-related preferences in TRBV/TRBJ usage and combinations, as well as lots of T cell clones shared by different groups involved in public T cell responses, which provide more detailed insights into the divergent disease evolution.
Epstein-Barr Virus Type 2 Infects T Cells and Induces B Cell Lymphomagenesis in Humanized Mice
An EBV-2 humanized mouse model is developed, utilizing immunodeficient mice engrafted with human cord blood CD34+ stem cells, that establishes a latency III infection with evidence of ongoing viral reactivation in both B and T cells and efficiently induces lymphomagenesis in humanized mice.
CD30-Positive T-Cell Lymphoproliferative Disease of the Oral Mucosa in Children: A Manifestation of Epstein-Barr Virus-Associated T-Lymphoproliferative Disorder
  • M. Hong, Y. Ko
  • Medicine
    Journal of pathology and translational medicine
  • 2015
Eosinophilic ulcer of the oral mucosa in children is a manifestation of EBV-positive T-cell LPD, and should therefore be distinguished from the disease in adults.


Immunophenotypic analysis of Epstein–Barr virus (EBV)‐infected CD8+ T cells in a patient with EBV‐associated hemophagocytic lymphohistiocytosis
Findings suggest that lacking expression of CD5 on CD8+ T cells with specific TCR VB may serve as a useful marker of dysregulated T‐cell activation and proliferation in EBV‐HLH.
Proposed categorization of pathological states of EBV‐associated T/natural killer‐cell lymphoproliferative disorder (LPD) in children and young adults: Overlap with chronic active EBV infection and infantile fulminant EBV T‐LPD
A clinicopathological categorization of EBV‐T/NK LPD is proposed, based on pathological evaluation and molecular data, and it is expected that this categorization system will provide a guide for the better understanding of this disorder.
EBV-Positive T/NK-Cell Lymphoproliferative Disease of Childhood
EBV-positive HLH and STLPD share similar clinicopathological findings and may constitute a continuous spectrum of acute EBV-associated T- or NK-cell proliferative disorders.
EBV-associated T and NK cell lymphoproliferative disorders: consensus report of the 4th Asian Hematopathology Workshop
The umbrella term “EBV-positive T/NK lymphoproliferative disease in childhood-type” covers the entire spectrum of EBV-associated lesions in childhood, ranging from reactive to neoplastic processes.
Massive expansion of EBV+ monoclonal T cells with CD5 down regulation in EBV-associated haemophagocytic lymphohistiocytosis
The patient responded dramatically to intravenous immunoglobulin, interferon α2b, ganciclovir and prednisolone, suggesting restoration of her immune system and eradication of the clonal T cells through these immunoregulatory agents.
Epstein-Barr virus-associated lymphoproliferative disease in non-immunocompromised hosts: a status report and summary of an international meeting, 8-9 September 2008.
The participants proposed an international consortium to facilitate further clinical and biological studies of novel EBV-driven LPDs to better define the pathogenesis, classification, and treatment of these disorders.
Aggressive Natural Killer Cell Leukemia: Is Epstein-Barr Virus Negativity an Indicator of a Favorable Prognosis?
EBV-negative AnKL is an uncommon malignancy that pursues a less aggressive clinical course than EBV-positive ANKL and has a longer survival than EBv-positive patients.
Hypersensitivity to mosquito bites as the primary clinical manifestation of a juvenile type of Epstein-Barr virus-associated natural killer cell leukemia/lymphoma.
It is proposed that this disease, defined as the triad of hypersensitivity to mosquito bites, chronic Epstein-Barr virus infection, and natural killer cell leukemia/lymphoma, is a clinical entity mostly seen in Asians.
Clinicopathological study of severe chronic active Epstein‐Barr virus infection that developed in association with lymphoproliferative disorder and/or hemophagocytic syndrome
A retrospective study on Epstein‐Barr virus patients found that the monoclonal dissemination of EBV‐infected cells develops from oligoclonals or polyclonal EBV­ infected cells, and these findings seem to be related with the causes of death due to hemophagocytic syndrome.