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
Contents
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[5]:510-511[4][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
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- ↑ 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.
