Plasmablastic lymphoma

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

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

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+, CD38+ and MUM1+.[2]:379 Ki67 is over 90%.[2]:378 EBV is positive in 75%; HHV-8 is negative.[2]:379

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. 1 2 3 4 5 6 7 8 9 10 11 Jaffe ES, Harris NL, Vardiman JW, Campo E, Arber, DA (2011). Hematopathology (1st ed.). Elsevier Saunders. ISBN 9780721600406.
  3. 1 2 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. PMID 25641215. doi:10.1586/17474086.2015.1007862.
  4. 1 2 3 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. PMC 4155383 Freely accessible. PMID 25206297. doi:10.3748/wjg.v20.i33.11894.
  5. ↑ Armitage, JO; Mauch PM; Harris NL; et al. (2010). "Chapter 35". Non-Hodgkin Lymphomas (2nd ed.). Lippincott Williams & Wilkins. ISBN 9780781791168.
  6. 1 2 Castillo JJ, Reagan JL (2011). "Plasmablastic lymphoma: a systematic review". ScientificWorldJournal. 11: 687–96. PMID 21442146. doi:10.1100/tsw.2011.59.
  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.
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