Epstein Barr virus positive diffuse large B-cell lymphoma, not otherwise specified

Epstein–Barr virus positive diffuse large B-cell lymphoma, not otherwise specified (also termed: EBV positive diffuse large B cell lymphoma, NOS; EBV+ DLBCL, NOS; or EBV+ DLBCL) was initially termed in the WHO 2008 classification as Epstein–Barr virus -positive DLBCL of the elderly because it was a specific type of large B-cell lymphoma that appeared to be limited to elderly (e.g. >50 years old) individuals.[1][2]:369–370[3] Since this 2008 WHO classification, however, the disorder has been diagnosed in much younger adults and children.[4] Accordingly, the WHO classification of 2016 renamed this order as EBV+ DLBCL, NOS.[5] The disease is also classified as one of numerous related and interrelated Epstein-Barr virus-associated lymphoproliferative diseases.[4]

EBV+ DLBCL, NOS is usually CD20 positive, and has clonal immunoglobulin gene rearrangement.[2]:380

Biology

This type of lymphoma is not associated with immunodeficiency.[2]:369 Although reported almost exclusively in Asians, it is not confined to that population.[2]:369[6] The disease usually has an extranodal presentation, with or without lymph node involvement.[2]:369

Morphologically, areas of necrosis are often seen[2]:370[3] as well as Reed–Sternberg-like cells.[2]:370[3] There are two subtypes: one with monotonous large cells, the other with numerous cell sizes as well as reactive cells, but different clinical behavior is not appreciated between these subtypes.[2]:370 Morphological differential diagnosis is Hodgkin lymphoma.[2]:370

Median survival 2 years, 25% 5-year survival.[2]:369

See also

References
  1. Swerdlow, Steven H.; International Agency for Research on Cancer; World Health Organization (2008). WHO classification of tumours of haematopoietic and lymphoid tissues. World Health Organization classification of tumours. 2 (4th ed.). International Agency for Research on Cancer. ISBN 9789283224310.
  2. Jaffe ES, Harris NL, Vardiman JW, Campo E, Arber, DA (2011). Hematopathology (1st ed.). Elsevier Saunders. ISBN 9780721600406.
  3. Adam P, Bonzheim I, Fend F, Quintanilla-Martínez L (2011). "Epstein-Barr virus-positive diffuse large B-cell lymphomas of the elderly". Adv Anat Pathol. 18 (5): 349–55. doi:10.1097/PAP.0b013e318229bf08. PMID 21841405.
  4. Dojcinov SD, Fend F, Quintanilla-Martinez L (March 2018). "EBV-Positive Lymphoproliferations of B- T- and NK-Cell Derivation in Non-Immunocompromised Hosts". Pathogens (Basel, Switzerland). 7 (1). doi:10.3390/pathogens7010028. PMC 5874754. PMID 29518976.
  5. Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R, Advani R, Ghielmini M, Salles GA, Zelenetz AD, Jaffe ES (May 2016). "The 2016 revision of the World Health Organization classification of lymphoid neoplasms". Blood. 127 (20): 2375–90. doi:10.1182/blood-2016-01-643569. PMC 4874220. PMID 26980727.
  6. Ok CY, Papathomas TG, Medeiros LJ, Young KH (2013). "EBV-positive diffuse large B-cell lymphoma of the elderly". Blood. 122 (3): 328–40. doi:10.1182/blood-2013-03-489708. PMC 3779382. PMID 23649469.
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