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Abstract
Chronic active Epstein-Barr virus infection (CAEBV) is a prototype of EBV-associated T- and/or NK-cell (EBV+ T/NK-cell) lymphoproliferative disorders. Most subtypes of these are lethal. We established a unified treatment strategy composed of step 1 (immunochemotherapy: steroids, cyclosporine A, and etoposide), step 2 (multi-drug block chemotherapy), and step 3 (allogeneic hematopoietic stem cell transplantation; HSCT) for CAEBV and its related diseases. Allogeneic HSCT is the only cure for CAEBV with few exceptions. Primary-EBV infection-associated hemophagocytic lymphohistiocytosis (primary-EBV HLH) is also an EBV+ T/NK-cell lymphoproliferation. The nature of EBV+ T/NK cells in CAEBV and those in primary-EBV HLH differ. In primary-EBV HLH, most patients need step 1 only and some require step 2 for the successful induction of apoptosis in EBV-infected T cells; however, some exceptional patients require HSCT. We herein present our single institutional experience of CAEBV and primary-EBV HLH, together with that of post-transplant EBV+ T/NK-cell lymphoproliferative disease. We also discuss some practical points on HCST with a review of the literature.
Highlights
Epstein-Barr virus (EBV), a B-cell lymphotropic virus, was revealed to have the potential to infect T and NK cells and cause lymphoproliferative diseases (LPDs) in 1988 and 1989 [1,2,3,4]
Primary-EBV HLH is a secondary HLH following a primary EBV infection; secondary means that it occurs in children without known immunodeficiencies, including familial HLH (FHL)
A systematic review and meta-analysis showed that survival ratios were 68 and 80% in the immunochemotherapy group and hematopoietic stem cell transplantation (HSCT) group, respectively, and concluded that both approaches contributed to decreasing mortality [36]; disease severity may differ in each group
Summary
Epstein-Barr virus (EBV), a B-cell lymphotropic virus, was revealed to have the potential to infect T and NK cells and cause lymphoproliferative diseases (LPDs) in 1988 and 1989 [1,2,3,4]. Allogeneic hematopoietic stem cell transplantation (HSCT) has been the most reliable radical treatment It has a mortality rate of ∼10%, and a severe morbidity rate of another 10%. The main targets of our current literature are CAEBV, its related diseases, and primary-EBV infectionassociated hemophagocytic lymphohistiocytosis (primary-EBV HLH); EBV+ T/NK-cell lymphomas or leukemia and post-transplant EBV+ T/NK-cell lymphoproliferative disease (T/NK-LPD) are referred. This retrospective study was approved by the Research Ethics Committee of Osaka Women’s and Children’s Hospital (the name has been changed: previously, Osaka Medical Center and Research Institute for Maternal and Child Health)
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