Plasmablastic lymphoma

Plasmablastic lymphoma is a type of large B-cell lymphoma, recognized in the WHO 2008 classification.[1][2]:378–380[3] It is CD20 negative, and has an immunophenotype that resembles plasma cells.[2]:378–380 In formal use, lymphomas with plasmablastic immunophenotype such as primary effusion lymphoma, ALK+ large B-cell lymphoma, large B-cell lymphoma arising in HHV8-associated multicentric Castleman's disease and extracavitary HHV–8-positive lymphoma are not part of this category, although sometimes the literature has confused this point.[2]:378–380

This article deals with plasmablastic lymphoma as defined in the WHO 2008 classification. For other entities sometimes termed plasmablastic lymphoma, see Large B-cell lymphoma arising in HHV8-associated multicentric Castleman's disease and Extracavitary HHV–8-positive lymphoma

Biology

The median age of diagnosis is approximately fourth and fifth decades.[2]:378 The disease often arises in the oral cavity, particularly in HIV disease,[4] but other sites include nasal cavity, gastrointestinal tract, skin, bone soft tissue, and lung.[2]:378[4][5]:510–511[6] There is usually immunodeficiency, such as HIV, organ transplants, autoimmune diseases[2]:378[7]

Like Burkitt's lymphoma, the morphology has a "starry sky".[2]:378 However, the immunophenotype resembles plasma cells: CD45-, CD20-, CD79a+/-, PAX5-, CD38+, CD138+ and MUM1+.[2]:379 Ki67 is over 90%.[2]:378 Epstein–Barr virus (EBV) is positive in 75%; HHV-8 is negative.[2]:379 The World Health Organization (2016) classified EBV-positive plasmablastic lymphoma as one of the Epstein-Barr virus-associated lymphoproliferative diseases, terming it Epstein Barr virus-positive plasmablastic lymphoma.[8]

Treatment

Chemotherapy with CHOP, infusional EPOCH, hyperCVAD, and CODOX-M/IVAC is often used.[4][6] The prognosis is generally poor,[3] for example 6 to 7 months[2]:378 and 14 months.[4]

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. Castillo JJ, Chavez JC, Hernandez-Ilizaliturri FJ, Montes-Moreno S (2015). "CD20-negative diffuse large B-cell lymphomas: biology and emerging therapeutic options". Expert Rev Hematol. 8: 1–12. doi:10.1586/17474086.2015.1007862. PMID 25641215.
  4. Luria L, Nguyen J, Zhou J, et al. (2014). "Manifestations of gastrointestinal plasmablastic lymphoma: a case series with literature review". World J. Gastroenterol. 20 (33): 11894–903. doi:10.3748/wjg.v20.i33.11894. PMC 4155383. PMID 25206297.
  5. Armitage, JO; Mauch PM; Harris NL; et al. (2010). "Chapter 35". Non-Hodgkin Lymphomas (2nd ed.). Lippincott Williams & Wilkins. ISBN 9780781791168.
  6. Castillo JJ, Reagan JL (2011). "Plasmablastic lymphoma: a systematic review". ScientificWorldJournal. 11: 687–96. doi:10.1100/tsw.2011.59. PMC 5720057. PMID 21442146.
  7. Acta Cytol. 2014;58(3):309-17. doi: 10.1159/000360359. Epub 2014 Mar 27. Extraoral plasmablastic lymphoma detected using ascitic fluid cytology and flow cytometry: a case report with a review of the literature.
  8. Rezk SA, Zhao X, Weiss LM (June 2018). "Epstein - Barr virus - associated lymphoid proliferations, a 2018 update". Human Pathology. doi:10.1016/j.humpath.2018.05.020. PMID 29885408.
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