International Journal of Hematology

, Volume 78, Issue 1, pp 24-31

First online:

Diagnosis and treatment of epstein-barr virus-associated natural killer cell lymphoproliferative disease

  • Keisei KawaAffiliated withDepartment of Pediatrics, Osaka Medical Center and Research Institute for Maternal and Child Health Email author 

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Epstein-Barr virus (EBV) exhibits tropism for both lymphocytes and epithelial cells and can induce both replicative (productive/ lytic) and latent (persistent) infections that result in a variety of human diseases.With regard to lymphocytes, latent EBV infection is linked to development of heterogeneous lymphoproliferative disease (LPD), such as B-cell LPD and T-cell/ natural killer cell (T/NK cell) LPD. Unlike B-cell LPD, LPD derived from T-cells and NK cells sometimes has overlapping clinical symptoms, as well as histologic and immunophenotypic features, because both types of cells are derived from a common precursor. However, determination of cell lineage is important in classification of lymphoid neoplasms, and combined modern techniques allows us to distinguish NK cell LPD from T-cell LPD in most instances. Because NK cell LPD seems to be heterogeneous in terms of clinical features, prognosis, and diagnosis and has a monoclonal or polyclonal (or oligoclonal) nature, this review attempts to clarify recent research and clinical findings and to establish diagnostic and therapeutic strategies.

Key words

EBV Latent infection Lymphoproliferative disease NK cell LPD Stem cell transplantation