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WHO Classification of Tumours
Systemic EBV+ T-cell lymphoproliferative disease of childhood
Tumours of haematopoietic and lymphoid tissues


Definition

Systemic EBV+ T-cell LPD of childhood is a life-threatening illness of children and young adults characterized by a clonal proliferation of EBV-infected T-cells with an activated cytotoxic phenotype. It can occur shortly after primary acute EBV infection or in the setting of chronic active EBV infection (CAEBV). It has a rapid progression with multiple organ failure, sepsis and death, usually from days to weeks. This entity shows some overlapping clinicopathologic features with aggressive NK-cell leukaemia.

> Synonyms and historical annotation
Historically this process has been described under a variety of terms including: fulminant EBV+ T-cell LPD of childhood, sporadic fatal infectious mononucleosis (FIM); fulminant haemophagocytic syndrome in children in Taiwan

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Su IJ, Chen RL, Lin DT, Lin KS, Chen CC (1994)
Epstein-Barr virus (EBV) infects T lymphocytes in childhood EBV-associated hemophagocytic syndrome in Taiwan.
Am J Pathol 144: 1219-25



; fatal EBV-associated haemophagocytic syndrome in Japan
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Kikuta H, Sakiyama Y, Matsumoto S, Oh-Ishi T, Nakano T, Nagashima T, Oka T, Hironaka T, Hirai K (1993)
Fatal Epstein-Barr virus-associated hemophagocytic syndrome.
Blood 82: 3259-64



; and severe CAEBV
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Kimura H, Hoshino Y, Kanegane H, Tsuge I, Okamura T, Kawa K, Morishima T (2001)
Clinical and virologic characteristics of chronic active Epstein-Barr virus infection.
Blood 98: 280-6




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Okano M, Matsumoto S, Osato T, Sakiyama Y, Thiele GM, Purtilo DT (1991)
Severe chronic active Epstein-Barr virus infection syndrome.
Clin Microbiol Rev 4: 129-35




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Suzuki K, Ohshima K, Karube K, Suzumiya J, Ohga S, Ishihara S, Tamura K, Kikuchi M (2004)
Clinicopathological states of Epstein-Barr virus-associated T/NK-cell lymphoproliferative disorders (severe chronic active EBV infection) of children and young adults.
Int J Oncol 24: 1165-74



. The term fulminant or fatal haemophagocytic syndrome has been used to describe a systemic disease secondary to acute primary EBV infection affecting previously healthy children. It has been shown to be a monoclonal CD8+ EBVassociated lymphoproliferative disorder, and therefore is now considered equivalent to systemic EBV+ T-cell LPD of childhood
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Quintanilla-Martinez L, Kumar S, Fend F, Reyes E, Teruya-Feldstein J, Kingma DW, Sorbara L, Raffeld M, Straus SE, Jaffe ES (2000)
Fulminant EBV(+) T-cell lymphoproliferative disorder following acute/chronic EBV infection: a distinct clinicopathologic syndrome.
Blood 96: 443-51



. The term CAEBV was coined to describe an infectious mononucleosis-like syndrome persisting for at least 6 months, and associated with high titers of antibodies to EBV-capsid antigen (VCA-IgG) and early antigen (EA-IgG) without association to malignancy, autoimmune diseases or immunodeficiency
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Straus SE (1988)
The chronic mononucleosis syndrome.
J Infect Dis 157: 405-12



. These cases are mostly seen in Western populations; progression to EBV-positive T-cell LPD has been reported rarely in such patients
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Jones JF, Shurin S, Abramowsky C, Tubbs RR, Sciotto CG, Wahl R, Sands J, Gottman D, Katz BZ, Sklar J (1988)
T-cell lymphomas containing Epstein-Barr viral DNA in patients with chronic Epstein-Barr virus infections.
N Engl J Med 318: 733-41




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Kanegane H, Bhatia K, Gutierrez M, Kaneda H, Wada T, Yachie A, Seki H, Arai T, Kagimoto S, Okazaki M, Oh-ishi T, Moghaddam A, Wang F, Tosato G (1998)
A syndrome of peripheral blood T-cell infection with Epstein-Barr virus (EBV) followed by EBV-positive T-cell lymphoma.
Blood 91: 2085-91




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Quintanilla-Martinez L, Kumar S, Fend F, Reyes E, Teruya-Feldstein J, Kingma DW, Sorbara L, Raffeld M, Straus SE, Jaffe ES (2000)
Fulminant EBV(+) T-cell lymphoproliferative disorder following acute/chronic EBV infection: a distinct clinicopathologic syndrome.
Blood 96: 443-51



. A more severe form of CAEBV characterized by high fever, hepatosplenomegaly, extensive lymphadenopathy, and pancytopenia has been described in Japan
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Kimura H, Hoshino Y, Kanegane H, Tsuge I, Okamura T, Kawa K, Morishima T (2001)
Clinical and virologic characteristics of chronic active Epstein-Barr virus infection.
Blood 98: 280-6




Click to access Pubmed
Kimura H, Morishima T, Kanegane H, Ohga S, Hoshino Y, Maeda A, Imai S, Okano M, Morio T, Yokota S, Tsuchiya S, Yachie A, Imashuku S, Kawa K, Wakiguchi H, (2003)
Prognostic factors for chronic active Epstein-Barr virus infection.
J Infect Dis 187: 527-33




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Suzuki K, Ohshima K, Karube K, Suzumiya J, Ohga S, Ishihara S, Tamura K, Kikuchi M (2004)
Clinicopathological states of Epstein-Barr virus-associated T/NK-cell lymphoproliferative disorders (severe chronic active EBV infection) of children and young adults.
Int J Oncol 24: 1165-74



. These patients have higher viral copy numbers in peripheral blood (PB), and instead of B-cells, T-cells or natural killer (NK)-cells are EBV-infected. These cases frequently manifest monoclonal T-cell populations and progression to overt malignant T-cell malignancy is the rule. CAEBV with monoclonal EBV+ T-cell proliferation represents part of the spectrum of systemic EBV+ T-cell LPD of childhood
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Quintanilla-Martinez L, Kumar S, Fend F, Reyes E, Teruya-Feldstein J, Kingma DW, Sorbara L, Raffeld M, Straus SE, Jaffe ES (2000)
Fulminant EBV(+) T-cell lymphoproliferative disorder following acute/chronic EBV infection: a distinct clinicopathologic syndrome.
Blood 96: 443-51



, and to avoid confusion should not be referred as to CAEBV.


> Related Topics
Introduction: EBV-positive T-cell lymphoproliferative disorders of childhood
Hydroa vacciniforme-like lymphoma
Introduction: Classification of lymphoid neoplasms