Definition
In addition to primary effusion lymphoma (PEL) (Cesarman et al. 1995) and its solid extracavitary variants (Chadburn et al. 2004), which are by definition Kaposi sarcoma-associated herpesvirus (KSHV)-associated lymphomas, the spectrum of KSHV-associated lymphoproliferative diseases has been expanded by the identification of cases of KSHV-associated extracavitary lymphomas without serous effusions (Carbone and Gloghini 2008). Moreover, in the HIV setting, KSHV is associated with multicentric Castleman disease (MCD) and another rare neoplastic lymphoproliferative disorder, namely, large B-cell lymphoma arising in KSHV-associated MCD. KSHV-infected B cells in MCD have a pre-plasma cell phenotype and plasmacytic/plasmablastic morphology (Bower 2010; Uldrick et al. 2012).
KSHV-associated lymphoid disorders are closely linked to HIV infection and, when MCD unrelated, may be associated to EBV infection of the...
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References and Further Reading
Bower, M. (2010). How I treat HIV-associated multicentric Castleman disease. Blood, 116, 4415–4421.
Carbone, A., & Gloghini, A. (2008). KSHV/HHV8-associated lymphomas. British Journal of Haematology, 140, 13–24.
Carbone, A., De Paoli, P., Gloghini, A., & Vaccher, E. (2015). KSHV-associated multicentric Castleman disease: A tangle of different entities requiring multitarget treatment strategies. International Journal of Cancer, 137, 251–261.
Cesarman, E., Chang, Y., Moore, P. S., Said, J. W., & Knowles, D. M. (1995). Kaposi’s sarcoma-associated herpesvirus-like DNA sequences in AIDS-related body-cavity-based lymphomas. New England Journal of Medicine, 332, 1186–1191.
Chadburn, A., Hyjek, E., Mathew, S., Cesarman, E., Said, J., & Knowles, D. M. (2004). KSHV-positive solid lymphomas represent an extra-cavitary variant of primary effusion lymphoma. American Journal of Surgical Pathology, 28, 1401–1416.
Polizzotto, M. N., Uldrick, T. S., & Yarchoan, R. (2014). Multicentric Castleman disease. In E. Yarchoan (Ed.), Cancers in people with HIV and AIDS (pp. 245–260). New York: Springer.
Uldrick, T. S., Polizzotto, M. N., & Yarchoan, R. (2012). Recent advances in Kaposi sarcoma herpesvirus-associated multicentric Castleman disease. Current Opinion in Oncology, 24, 495–505.
Medeiros, L.J, O’Malley, D.P., Caraway, N.P., Vega, F., Elenitoba-Johnson, K.S.J. & Lim, M.S. (2017) Human herpesvirus 8-positive large B-cell lymphomas and associated diseases. In: Tumors of the lymph nodes and spleen (pp 363–374) AFIP Atlas of Tumor Pathology, 4th Series, Fascicle 25. Washington DC: American Registry of Pathology.
Said, J, Isaacson, P.G., Campo, E. & Harris, N.L. (2017) HHV-8 associated lymphoproliferative disorders. In Swerdlow, S.H., Campo, E., Harris, N.L., Jaffe, E.S., Pileri, S.A., Stein, H., Thiele, J., Arber, T.A., Hasserjian, R.P., Le Beau, M.M., Orazi, A. & Siebert R. eds. WHO classification of tumours of haematopoietic and lymphoid tissues (pp 325–329) Revised 4th ed. Lyon: IARC.
Yarchoan, R. & Uldrick, T.S. (2018) HIV-Associated Cancers and Related Diseases. New England Journal of Medicine, 378, 1029–1041.
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Carbone, A., Gloghini, A. (2018). KSHV-Associated Lymphoid Disorders. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_3832-1
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