{"entity":{"id":"plasmablastic-lymphoma","kind":"cancer","name":"Plasmablastic lymphoma","aka":["PBL","Plasmablastic lymphoma of the oral cavity","Plasmablastic lymphoma, HIV-associated"],"tldr":"An aggressive lymphoma whose cells have taken on the appearance of plasma cells, so they no longer carry the CD20 marker that most B-cell lymphoma treatments aim at. It most often starts in the mouth or jaw, and about half of people diagnosed with it have HIV.","summary":"What it is. A large B-cell lymphoma whose cells have travelled most of the way to becoming plasma cells, the antibody factories that B cells turn into at the end of their life. That matters practically rather than philosophically: a plasma cell has switched off CD20, so rituximab and the antibody treatments that follow it cannot see the tumour. Instead the cells carry plasma cell markers, CD138 and MUM1, and the diagnosis is made on that pattern together with a very high proliferation index.\n\nWho gets it. In the largest United States analysis, 1,153 patients in SEER and 1,822 in the National Cancer Database diagnosed between 2010 and 2020, the incidence was 0.07 cases per 100,000 people a year, 77 per cent were men, and half had HIV. In an earlier SEER analysis of 248 treated patients, 82 per cent were men and 71 per cent were under 60, with the mouth and the gastrointestinal tract the commonest starting points at 23 and 19 per cent. It also occurs after organ transplant and in older people with age-related decline in immunity.\n\nThe site matters. Disease starting in the mouth was associated with better survival in the SEER analysis, and with less likelihood of advanced stage and of fevers and weight loss. That is a real signal and not only a statistical one: a lump on the gum or in the jaw is noticed early.\n\nWhat the figures say, and why two of them disagree. The United Kingdom population series that reports lymphoma by subtype found 24 cases among 5,796 lymphomas diagnosed between 2004 and 2012, an age-standardised rate of 0.07 per 100,000, with a five-year relative survival of 17.2 per cent. The United States analysis of patients treated between 2010 and 2020 reported median overall survival of 58.6 months among those who received multi-agent chemotherapy. Those two numbers describe different things: the first is everybody diagnosed in an earlier decade, the second is the subset who were well enough to be treated with combination chemotherapy in a later one. Both are quoted here because quoting only the second would flatter the disease and quoting only the first would date it.\n\nHow it is treated. More intensively than ordinary diffuse large B-cell lymphoma, because standard immunochemotherapy without rituximab does less well, and with antiretroviral therapy where there is HIV; HIV status did not affect survival in either of the United States analyses once treatment was given. Many cases carry a MYC rearrangement. Plasma-cell directed drugs such as bortezomib and lenalidomide have been added in series on the logic of the phenotype rather than on randomised evidence. The regimens are in the treatment layer of this family and on the HIV-associated lymphoma page.","asOf":"2026-09-29","wikipedia":"https://en.wikipedia.org/wiki/Plasmablastic_lymphoma","links":[{"label":"WHO Classification of Haematolymphoid Tumours, 5th edition: lymphoid neoplasms (Alaggio, Leukemia 2022)","url":"https://doi.org/10.1038/s41375-022-01620-2"},{"label":"International Consensus Classification of Mature Lymphoid Neoplasms (Campo, Blood 2022)","url":"https://doi.org/10.1182/blood.2022015851"},{"label":"Epidemiologic characteristics, treatment patterns and survival of plasmablastic lymphoma in the United States, a SEER and NCDB analysis (Clinical Lymphoma Myeloma and Leukemia 2024)","url":"https://doi.org/10.1016/j.clml.2023.12.014"},{"label":"Survival analysis in treated plasmablastic lymphoma patients, a population-based study of 248 patients (American Journal of Hematology 2020)","url":"https://doi.org/10.1002/ajh.25955"},{"label":"Lymphoma incidence, survival and prevalence 2004 to 2014, subtype analyses from the UK Haematological Malignancy Research Network (Smith, Br J Cancer 2015)","url":"https://doi.org/10.1038/bjc.2015.94"}],"tags":["heme","lymphoma","subtype-page"],"related":["hiv-associated-lymphoma","dlbcl","multiple-myeloma","primary-effusion-lymphoma","non-hodgkin-lymphoma"],"cancers":[],"sections":[],"technologies":["histopathology-ihc","fdg-pet"],"targets":["myc"],"drugs":["bortezomib","lenalidomide"],"companies":[],"institutions":[],"pathways":[],"terms":["ebv-term","lymphoma-b-versus-t-cell","lymphoma-classification-2022","lymphoma-nodal-versus-extranodal"],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"haematologic","burden":"An incidence of 0.07 cases per 100,000 people a year in the United States, from the largest analysis to date (1,153 patients in SEER and 1,822 in the National Cancer Database, 2010 to 2020); 77 per cent of patients are men and half have HIV. In the United Kingdom population series, 24 of 5,796 lymphomas diagnosed between 2004 and 2012, an age-standardised rate of 0.07 per 100,000 and a median age of 70.9 years.","subtypes":["HIV-associated plasmablastic lymphoma","Post-transplant and other immune-deficiency-associated plasmablastic lymphoma","Plasmablastic lymphoma in an immunocompetent person, usually older"],"biomarkers":["A plasma cell phenotype: CD138 and MUM1 positive, CD20 and PAX5 usually negative, which is why anti-CD20 antibodies do not work","A very high Ki-67 proliferation index, usually above 80 per cent","Epstein-Barr virus by EBER in situ hybridisation, positive in most HIV-associated cases","MYC rearrangement, found in a large proportion","HIV status, which is positive in about half of patients and should be tested in every one"],"standardOfCare":[{"setting":"Making the diagnosis, and why it is missed","approach":"The cells look like plasma cells and the surface markers agree with that appearance, so the differential diagnosis includes myeloma with plasmablastic features rather than other lymphomas. What separates them is the clinical picture: a rapidly growing mass, usually in the mouth or jaw or the gut, in a younger person, often with HIV, with Epstein-Barr virus in the cells and a very high proliferation index. HIV testing belongs in every work-up.","refs":["histopathology-ihc","ebv-term","multiple-myeloma","myc"],"guideline":{"version":"WHO Classification of Haematolymphoid Tumours, 5th edition (2022), with the International Consensus Classification (2022) where they differ","url":"https://doi.org/10.1038/s41375-022-01620-2"}},{"setting":"Treatment","approach":"Combination chemotherapy more intensive than standard immunochemotherapy, because there is no CD20 for rituximab to attach to, together with antiretroviral therapy where there is HIV. In the two large United States analyses, HIV status did not affect survival once treatment was given. Drugs used in myeloma, such as bortezomib and lenalidomide, have been added in case series on the basis of the phenotype rather than on randomised evidence. The regimens are in the treatment layer of this family and on the HIV-associated lymphoma page.","refs":["hiv-associated-lymphoma","bortezomib","lenalidomide","cyclophosphamide","doxorubicin","etoposide","lymphoma-tx-regimen-alphabet"],"guideline":{"version":"NCI PDQ: adult non-Hodgkin lymphoma treatment","url":"https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq"}}],"stateOfArt":[],"history":[{"year":1997,"title":"Described in the mouths of people with HIV","note":"The first series described an aggressive lymphoma of the oral cavity in people with HIV whose cells had a plasma cell phenotype and did not carry CD20.","refs":[]},{"year":2020,"title":"A population picture of treated patients","note":"Among 248 patients treated with chemotherapy in the SEER registries between 2010 and 2016, three-year overall survival was 54 per cent, and disease starting in the mouth carried better survival than other sites.","refs":["hiv-associated-lymphoma"]},{"year":2024,"title":"The largest analysis, and HIV status not a predictor","note":"Across 1,153 SEER and 1,822 National Cancer Database patients, incidence was 0.07 per 100,000 a year, median overall survival among those given multi-agent chemotherapy was 58.6 months, and HIV status had no significant effect on survival.","refs":[]}],"pipeline":[],"openProblems":["No randomised trial has been run in plasmablastic lymphoma, and the regimens in use are chosen by analogy with other aggressive lymphomas and with myeloma.","There is no surface target. Every advance in B-cell lymphoma since 1997 has depended on CD20 or CD19, and this disease expresses neither reliably.","Half of patients have HIV, and the disease is a common first presentation of undiagnosed infection, so part of the burden belongs to HIV testing rather than to oncology."],"parent":"non-hodgkin-lymphoma"},"route":"/cancers/plasmablastic-lymphoma/","neighbours":{"cancer":[{"id":"dlbcl","kind":"cancer","name":"Diffuse large B-cell lymphoma","route":"/cancers/dlbcl/"},{"id":"hiv-associated-lymphoma","kind":"cancer","name":"HIV-associated (AIDS-related) lymphomas","route":"/cancers/hiv-associated-lymphoma/"},{"id":"multiple-myeloma","kind":"cancer","name":"Multiple myeloma","route":"/cancers/multiple-myeloma/"},{"id":"non-hodgkin-lymphoma","kind":"cancer","name":"Non-Hodgkin lymphoma (all types)","route":"/cancers/non-hodgkin-lymphoma/"},{"id":"primary-effusion-lymphoma","kind":"cancer","name":"Primary effusion lymphoma","route":"/cancers/primary-effusion-lymphoma/"}],"technology":[{"id":"fdg-pet","kind":"technology","name":"FDG PET","route":"/technologies/fdg-pet/"},{"id":"histopathology-ihc","kind":"technology","name":"Histopathology & immunohistochemistry","route":"/technologies/histopathology-ihc/"}],"pathway":[{"id":"myc","kind":"pathway","name":"MYC","route":"/pathways/myc/"}],"drug":[{"id":"bortezomib","kind":"drug","name":"Bortezomib","route":"/drugs/bortezomib/"},{"id":"cyclophosphamide","kind":"drug","name":"Cyclophosphamide","route":"/drugs/cyclophosphamide/"},{"id":"doxorubicin","kind":"drug","name":"Doxorubicin","route":"/drugs/doxorubicin/"},{"id":"etoposide","kind":"drug","name":"Etoposide","route":"/drugs/etoposide/"},{"id":"lenalidomide","kind":"drug","name":"Lenalidomide","route":"/drugs/lenalidomide/"}],"term":[{"id":"lymphoma-b-versus-t-cell","kind":"term","name":"B-cell, T-cell and NK-cell lymphoma","route":"/terms/lymphoma-b-versus-t-cell/"},{"id":"ebv-term","kind":"term","name":"Epstein-Barr virus (EBV) in cancer","route":"/terms/ebv-term/"},{"id":"lymphoma-nodal-versus-extranodal","kind":"term","name":"Nodal and extranodal lymphoma","route":"/terms/lymphoma-nodal-versus-extranodal/"},{"id":"lymphoma-tx-regimen-alphabet","kind":"term","name":"The lymphoma regimen alphabet: R-CHOP, pola-R-CHP, DA-EPOCH-R, ABVD, BEACOPP and the rest","route":"/terms/lymphoma-tx-regimen-alphabet/"},{"id":"lymphoma-classification-2022","kind":"term","name":"The two lymphoma classifications of 2022 (WHO-HAEM5 and ICC)","route":"/terms/lymphoma-classification-2022/"}]}}