Synonyms
Fatal EBV-associated hemophagocytic syndrome; Fulminant EBV-positive T-cell lymphoproliferative disorder of childhood; Fulminant hemophagocytic syndrome in children; Severe chronic active EBV infection; Sporadic fatal infectious mononucleosis
Definition
Systemic EBV-positive T-cell lymphoma 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 rapid progression, with multiorgan failure, sepsis, and death, usually within a timeframe of days to weeks. A hemophagocytic syndrome is nearly always present. This entity has some clinicopathological features overlapping with those of aggressive NK-cell leukemia (Quintanilla-Martinez et al. 2017).
Clinical Features
Incidence
Systemic EBV-positive T-cell lymphoma of childhood occurs most often in...
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References and Further Reading
Kikuta, H., Sakiyama, Y., Matsumoto, S., et al. (1993). Fatal Epstein-Barr virus-associated hemophagocytic syndrome. Blood, 82, 3259–3264.
Kimura, H., Hoshino, Y., Kanegane, H., et al. (2001). Clinical and virologic characteristics of chronic active Epstein-Barr virus infection. Blood, 98, 280–286.
Kimura, H., Ito, Y., Kawabe, S., et al. (2012). EBV-associated T/NK-cell lymphoproliferative diseases in nonimmunocompromised hosts: Prospective analysis of 108 cases. Blood, 119, 673–686.
Ohshima, K., Kimura, H., Yoshino, T., et al. (2008). Proposed categorization of pathological states of EBV-associated T/natural killer-cell lymphoproliferative disorder (LPD) in children and young adults: Overlap with chronic active EBV infection and infantile fulminant EBV T-LPD. Pathology International, 58, 209–217.
Quintanilla-Martinez, L., Kumar, S., Fend, F., et al. (2000). Fulminant EBV(+) T-cell lymphoproliferative disorder following acute/chronic EBV infection: A distinct clinicopathologic syndrome. Blood, 96, 443–451.
Quintanilla-Martinez, L., Ko, Y.-H., Kimura, H., et al. (2017). EBV-positive T-cell and NK-cell lymphoproliferative disease of childhood. In S. H. Swerdlow, E. Campo, N. L. Harris, et al. (Eds.), WHO classification of tumours of haematopoietic and lymphoid tissues (4th ed., pp. p355–p357). Lyon: IARC.
Smith, M. C., Cohen, D. N., Greig, B., et al. (2014). The ambiguous boundary between EBV-related hemophagocytic lymphohistiocytosis and systemic EBV-driven T cell lymphoproliferative disorder. International Journal of Clinical and Experimental Pathology, 7, 5738–5749.
Su, I. J., Chen, R. L., Lin, D. T., et al. (1994). Epstein-Barr virus (EBV) infects T lymphocytes in childhood EBV-associated hemophagocytic syndrome in Taiwan. The American Journal of Pathology, 144(6), 1219–1225.
Suzuki, K., Ohshima, K., Karube, K., et al. (2004). Clinicopathological states of Epstein-Barr virus-associated T/NK-cell lymphoproliferative disorders (severe chronic active EBV infection) of children and young adults. International Journal of Oncology, 24, 1165–1174.
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Nakazawa, A. (2020). Systemic EBV-Positive T-Cell Lymphoma of Childhood. In: Molina, T.J. (eds) Hematopathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-95309-0_4690
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DOI: https://doi.org/10.1007/978-3-319-95309-0_4690
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