An Adult Case of Severe Chronic Active Epstein-Barr Virus Infection Syndrome.

Yamada, Hiroshi; Kohno, Shigeru; Koga, Hironobu; Omagari, Katsuhisa; Maesaki, Shigefumi; Kaku, Mitsuo; Komatsu, Kazutoshi; Hara, Kohei

doi:10.2169/internalmedicine.31.1381

Cited by 6 publications

(3 citation statements)

References 16 publications

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“…No immune deficiency was detected, but virus-specific, cytotoxic T lymphocyte studies have not been done. NK cell number and function were normal in our patient, unlike in many other reported cases [32][33][34], which also is in keeping with the relatively low number ofEBV-containing mononuclear cells detected in the patient's peripheral blood.…”

Section: Discussionsupporting

confidence: 85%

Periodic Illness Associated with Epstein-Barr Virus Infection

Himes

Dale²,

Kingma³

et al. 1996

Clinical Infectious Diseases

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A lS-year-old boy with a 13-year history of periodic fevers, lymphadenopathy, and leukocytosis showed virological, serological, immunohistologic, and molecular evidence of persistent, active, Epstein-Barr virus (EBV) infection. Acyclovir and several other agents failed to alter his clinical course. Comprehensive immunological studies could not identify a defined immune deficiency syndrome to explain the persistent infection, although he does continue to have circulating polymeric EBV-specificimmunoglobulin type A, as is seen in individuals during acute EBV infections. In vitro work suggests that this polymeric antibody prevents B cell infection by EBV. Cumulative data suggest that this patient suffers from a novel form of EBV infection.

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Section: Discussionsupporting

confidence: 85%

Periodic Illness Associated with Epstein-Barr Virus Infection

Himes

Dale²,

Kingma³

et al. 1996

Clinical Infectious Diseases

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“… 12 , 13 However, similar clinical conditions have also been reported in adults (EBV-T/NK-LPD with CAEBV-like features in adult; adult-onset CAEBV). 14 , 15 …”

Section: Introductionmentioning

confidence: 99%

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

et al. 2017

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The characteristics of adult patients with chronic active Epstein-Barr virus infection are poorly recognized, hindering early diagnosis and an improved prognosis. We studied 54 patients with adult-onset chronic active Epstein-Barr virus infection diagnosed between 2005 and 2015. Adult onset was defined as an estimated age of onset of 15 years or older. To characterize the clinical features of these adults, we compared them to those of 75 pediatric cases (estimated age of onset <15 years). We compared the prognosis of adult-onset chronic active Epstein-Barr virus infection with that of patients with nasal-type (n=37) and non-nasal-type (n=45) extranodal NK/T-cell lymphoma. The median estimated age of onset of these lymphomas was 39 years (range, 16–86 years). Compared to patients with pediatric-onset disease, those in whom the chronic active Epstein-Barr virus infection developed in adulthood had a significantly decreased incidence of fever (P=0.005), but greater frequency of skin lesions (P<0.001). Moreover, hypersensitivity to mosquito bites and the occurrence of hydroa vacciniforme were less frequent in patients with adult-onset disease (P<0.001 and P=0.0238, respectively). Thrombocytopenia, high Epstein-Barr virus nuclear antigen antibody titer, and the presence of hemophagocytic syndrome were associated with a poor prognosis (P=0.0087, P=0.0236, and P=0.0149, respectively). Allogeneic hematopoietic stem cell transplantation may improve survival (P=0.0289). Compared to pediatric-onset chronic active Epstein-Barr virus infection and extranodal NK/T-cell lymphoma, adult-onset chronic active Epstein-Barr virus infection had a poorer prognosis (P<0.001 and P=0.0484, respectively). Chronic active Epstein-Barr virus infection can develop in a wide age range, with clinical differences between adult-onset and pediatric-onset disease. Adult-onset chronic active Epstein-Barr virus infection is a disease with a poor prognosis. Further research will be needed.

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“…Moreover, hepatic sinusoidal lymphocytosis also occurs in chronic infections with EpsteinBarr and hepatitis C viruses, and Felty's syndrome and is therefore a non-specific finding. [27][28][29] Despite these diagnostic difficulties it is crucial to make the correct diagnosis as HMS responds well to malaria prophylaxis whereas SLVL, particularly if symptomatic, may require splenectomy. 30 The criteria for HMS were established before SLVL was described and because of the reasons outlined we have found that they are not sufficiently stringent to distinguish between these two conditions.…”

Section: Tropical 'Big Spleen' Disease -The Role Of Malariamentioning

confidence: 99%

Chronic malaria and splenic lymphoma: clues to understanding lymphoma evolution

Bates

Bedu-Addo

1997

Leukemia

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In west Africa, splenic lymphoma with villous lymphocytes and hyper-reactive malarial splenomegaly have many clinical and immunological features in common suggesting an aetiopathological link. We hypothesize that in hyper-reactive malarial splenomegaly the dysregulated immune response to repeated malaria infections results in a stimulated, proliferating pool of B cells in which perturbation of cell growth and apoptosis by environmental and other factors promotes the development of SLVL. In Africa these factors are likely to include infectious agents several of which have already been associated with Bcell non-Hodgkin's lymphomas.

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An Adult Case of Severe Chronic Active Epstein-Barr Virus Infection Syndrome.

Cited by 6 publications

References 16 publications

Periodic Illness Associated with Epstein-Barr Virus Infection

Periodic Illness Associated with Epstein-Barr Virus Infection

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

Chronic malaria and splenic lymphoma: clues to understanding lymphoma evolution

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