A distinct subtype of Epstein-Barr virus-positive T/NK-cell lymphoproliferative disorder: adult patients with chronic active Epstein-Barr virus infection-like features

Kawamoto, Keisuke; Miyoshi, Hiroaki; Suzuki, Takaharu; Kozai, Yasuji; Katô, Kôji; Miyahara, Masaharu; Yujiri, Toshiaki; Choi, Ilseung; Fujimaki, Katsumichi; Muta, Tsuyoshi; Kume, Masaaki; Moriguchi, Sayaka; Tamura, Shinobu; Kato, Takumi; Tagawa, Hiroyuki; Makiyama, Junya; Kanisawa, Yuji; Sasaki, Yuya; Kurita, Daisuke; Yamada, Kyohei; Shimono, Joji; Sone, Hirohito; Takizawa, Jun; Seto, Masao; Kimura, Hiroshi; Ohshima, Koichi

doi:10.3324/haematol.2017.174177

Cited by 49 publications

(39 citation statements)

References 42 publications

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“…Some patients rapidly develop severe complications, such as multi-organ dysfunction and malignant lymphomas, whereas others remain stable without therapeutic intervention (12). Initially, CAEBV was thought to mainly be a disease of childhood, but, recently, increasing numbers of adult cases have been identified with slightly different clinical features compared to those of pediatric cases (13, 14).…”

Section: Definitions and Brief Description Of Representative Ebv Disementioning

confidence: 99%

Overview of EBV-Associated T/NK-Cell Lymphoproliferative Diseases

Kimura

Fujiwara

2019

Front. Pediatr.

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Epstein-Barr virus-associated T/natural killer-cell lymphoproliferative diseases (EBV-T/NK-LPDs) are a group of rare diseases resulting from ectopic infection of T or natural killer (NK) lymphocytes with Epstein-Barr virus (EBV). EBV-T/NK-LPDs include chronic active EBV infection, EBV-associated hemophagocytic lymphohistiocytosis, hydroa vacciniforme-like lymphoproliferative disease, and severe mosquito bite allergy. Extra-nodal NK/T-cell lymphoma-nasal type and aggressive NK-cell leukemia can also be included in this broad spectrum. Currently, the etiology of EBV-T/NK-LPDs is unknown and no curative therapy has been established, except for hematopoietic stem cell transplantation. While most cases of EBV-T/NK-LPDs have been documented in specific areas of the world, they have also been documented more broadly across East Asia and Latin America. Consequently, active research and discussion of EBV-T/NK-LPDs are both necessary and important within the extensive international community of scientists and clinicians, to elucidate their etiology and develop a standard therapy.

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Section: Definitions and Brief Description Of Representative Ebv Disementioning

confidence: 99%

Overview of EBV-Associated T/NK-Cell Lymphoproliferative Diseases

Kimura

Fujiwara

2019

Front. Pediatr.

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“…Half of the children (including adolescents) with CAEBV died in 5 years, and most of them died in 10–15 years without radical treatment ( 10 ). Two studies reported that adult-onset CAEBV progresses rapidly, and most of patients died within 5 years ( 22 , 23 ). In our series, 3y-OS was equivalent between adults (≥ 20 years of age at onset) and children (71.4 ± 12.1, 76.6 ± 5.3%, respectively; p = 0.61) ( 6 ).…”

Section: Hsct For Caebv In Various Situationsmentioning

confidence: 99%

Hematopoietic Stem Cell Transplantation for the Treatment of Epstein-Barr Virus-Associated T- or NK-Cell Lymphoproliferative Diseases and Associated Disorders

Sawada

Inoue

2018

Front. Pediatr.

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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.

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“…However, the report mentioned an increase in the number of adult patients. There are some differences between the childhood-onset and adult-onset disease with respect to the clinical features and prognosis [8,9]. Indeed, these may well be different disorders.…”

Section: Epidemiology Of Caebvmentioning

confidence: 99%

Chronic active Epstein–Barr virus infection: a bi-faceted disease with inflammatory and neoplastic elements

Arai

2018

Immunological Medicine

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Chronic active Epstein-Barr virus infection (CAEBV) is one of the Epstein-Barr virus (EBV)positive T-or NK-cell lymphoproliferative diseases. It is characterized by clonal proliferation of EBV-infected T or NK cells and their infiltration into systemic organs, leading to their failure. Inflammatory symptoms, fever, lymphadenopathy and liver dysfunction are main clinical findings of CAEBV. EBV itself contributes to the survival of the host cells via induction of CD40 and CD137 expression and constitutive activation of NF-jB. Accumulation of gene mutations in the infected cells may lead to the development of highly malignant lymphoma or leukemia. Furthermore, constitutive activation of STAT3 is detected in the infected cells, which not only promotes cell survival but also enhances production of inflammatory cytokines. Currently, allogeneic hematopoietic stem cell transplantation (allo-HSCT) is the only effective treatment strategy for eradication of EBV-infected T or NK cells. However, active disease at the time of allo-HSCT (defined as presence of fever, liver dysfunction, progressive skin lesions, vasculitis or uveitis) is a negative prognostic factor. Establishment of chemotherapy regimens for effective resolution of disease activity in patients with CAEBV is a key imperative. Based on the recently unraveled molecular mechanisms CAEBV development, pathways mediated by NF-jB or JAK/STAT are potential novel therapeutic targets.

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A distinct subtype of Epstein-Barr virus-positive T/NK-cell lymphoproliferative disorder: adult patients with chronic active Epstein-Barr virus infection-like features

Cited by 49 publications

References 42 publications

Overview of EBV-Associated T/NK-Cell Lymphoproliferative Diseases

Overview of EBV-Associated T/NK-Cell Lymphoproliferative Diseases

Hematopoietic Stem Cell Transplantation for the Treatment of Epstein-Barr Virus-Associated T- or NK-Cell Lymphoproliferative Diseases and Associated Disorders

Chronic active Epstein–Barr virus infection: a bi-faceted disease with inflammatory and neoplastic elements

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