Epstein–Barr virus positive diffuse large B-cell lymphoma
Epstein–Barr virus positive diffuse large B-cell lymphoma, not otherwise specified (EBV+ DLBCL, NOS) is a form of diffuse large B-cell lymphomas (DLBCL) accounting for around 10-15% of DLBCL cases. DLBCL are lymphomas in which B-cell lymphocytes proliferate excessively, invade multiple tissues, and often causes life-threatening tissue damage. EBV+ DLBCL is distinguished from DLBCL in that virtually all the large B cells in the tissue, infiltrates of the Epstien-Barr virus (EBV) express EBV genes characteristic of the virus's latency III (common in the elderly) or II (common in younger patients) phase.[2] EBV is a ubiquitous virus, infecting around 95% of the world population.
| Epstein–Barr virus positive diffuse large B-cell lymphoma | |
|---|---|
| Other names | EBV+ DLBCL, NOS |
| Specialty | Hematology, oncology |
| Symptoms | B symptoms |
| Causes | Epstein-Barr virus infection |
| Risk factors | Old age; immunosuppression |
| Diagnostic method | Histology of involved tissue |
| Treatment | Chemotherapy (R-CHOP) |
| Medication | Rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone, etoposide, prednisolone |
| Prognosis |
|
| Frequency | 5-15% of DLBCL cases |
EBV+ DLBCL, NOS was previously named Epstein–Barr virus-positive DLBCL of the elderly, by the WHO in 2008; because it appeared to be limited to people over the age of 50.[3][4]: 369–370 [5] The name was changed to EBV+ DLBCL, NOS by the WHO in 2016, after the disease was described in much younger adults and children.[6][7] The disease is also classified as one of numerous related and interrelated Epstein-Barr virus-associated lymphoproliferative diseases.[6] EBV+ DLBCL, NOS is usually CD20 positive, and has clonal immunoglobulin gene rearrangement.[4]: 380
Biology
editAlthough reported almost exclusively in Asians, it is not confined to that population.[4]: 369 [8] The disease usually has an extranodal presentation, with or without lymph node involvement.[4]: 369
Morphologically, areas of necrosis are often seen[4]: 370 [5] as well as Reed–Sternberg-like cells.[4]: 370 [5] 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.[4]: 370 Morphological differential diagnosis is Hodgkin lymphoma.[4]: 370 Median survival is 2 years in the elderly.[4]: 369
See also
editReferences
edit- ^ Bourbon, Estelle; Maucort-Boulch, Delphine; Fontaine, Juliette; Mauduit, Claire; Sesques, Pierre; Safar, Violaine; Ferrant, Emmanuelle; Golfier, Camille; Ghergus, Dana; Karlin, Lionel; Lazareth, Anne; Bouafia, Fadhela; Pica, Gian Matteo; Orsini-Piocelle, Frédérique; Rocher, Clément (2021-08-24). "Clinicopathological features and survival in EBV-positive diffuse large B-cell lymphoma not otherwise specified". Blood Advances. 5 (16): 3227–3239. doi:10.1182/bloodadvances.2021004515. ISSN 2473-9529. PMC 8405194. PMID 34427583.
- ^ Vockerodt M, Yap LF, Shannon-Lowe C, Curley H, Wei W, Vrzalikova K, Murray PG (January 2015). "The Epstein-Barr virus and the pathogenesis of lymphoma". The Journal of Pathology. 235 (2): 312–22. doi:10.1002/path.4459. PMID 25294567. S2CID 22313509.
- ^ 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. Vol. 2 (4th ed.). International Agency for Research on Cancer. ISBN 9789283224310. Archived from the original on October 12, 2013.
- ^ a b c d e f g h i Jaffe ES, Harris NL, Vardiman JW, Campo E, Arber, DA (2011). Hematopathology (1st ed.). Elsevier Saunders. ISBN 9780721600406.
- ^ a b c 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. S2CID 21320902.
- ^ a b 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): 28. doi:10.3390/pathogens7010028. PMC 5874754. PMID 29518976.
- ^ 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.
- ^ 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.