Plasmablastic lymphoma

Plasmablastic lymphoma is a type of large B-cell lymphoma, recognized in the WHO 2008 classification.:378–380 It is CD20 negative, and has an immunophenotype that resembles plasma cells.: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.:378–380 Plasmablastic lymphoma is a type of large B-cell lymphoma, recognized in the WHO 2008 classification.:378–380 It is CD20 negative, and has an immunophenotype that resembles plasma cells.: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.:378–380 The median age of diagnosis is approximately fourth and fifth decades.:378 The disease often arises in the oral cavity, particularly in HIV disease, but other sites include nasal cavity, gastrointestinal tract, skin, bone soft tissue, and lung.:378:510–511 There is usually immunodeficiency, such as HIV, organ transplants, autoimmune diseases:378 Like Burkitt's lymphoma, the morphology has a 'starry sky'.:378 However, the immunophenotype resembles plasma cells: CD45-, CD20-, CD79a+/-, PAX5-, CD38+, CD138+ and MUM1+.:379 Ki67 is over 90%.:378 Epstein–Barr virus (EBV) is positive in 75%; HHV-8 is negative.: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. Chemotherapy with CHOP, infusional EPOCH, hyperCVAD, and CODOX-M/IVAC is often used. The prognosis is generally poor, for example 6 to 7 months:378 and 14 months.