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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.
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.
Who 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.
The 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.
What 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.
How 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.
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.
Leukaemias, myeloma and MDS live in the marrow and blood; lymphomas grow in lymph nodes and spleen. The node stations are the disease map, not a route of spread, and staging counts them.
In lymphoma the node stations are the disease itself; staging (Ann Arbor / Lugano) counts how many regions and sides of the diaphragm are involved.
Same organ: High-grade B-cell lymphoma with MYC and BCL2 rearrangements (double-hit lymphoma), Mediastinal grey zone lymphoma, Primary effusion lymphoma, T-cell/histiocyte-rich large B-cell lymphoma, EBV-positive diffuse large B-cell lymphoma, Primary large B-cell lymphoma of the testis, Gastric MALT lymphoma, Ocular adnexal MALT lymphoma, Extranodal NK/T-cell lymphoma, Adult T-cell leukaemia/lymphoma, ALK-positive anaplastic large cell lymphoma, ALK-negative anaplastic large cell lymphoma, Breast implant-associated anaplastic large cell lymphoma, Primary cutaneous anaplastic large cell lymphoma, Lymphomatoid papulosis, Mycosis fungoides, Enteropathy-associated T-cell lymphoma, Monomorphic epitheliotropic intestinal T-cell lymphoma, Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), Splenic marginal zone lymphoma, Nodal marginal zone lymphoma, Primary cutaneous marginal zone lymphoma, Primary cutaneous follicle centre lymphoma, Sezary syndrome, Nodal T-follicular helper cell lymphoma, angioimmunoblastic type (angioimmunoblastic T-cell lymphoma), Hepatosplenic T-cell lymphoma, Intravascular large B-cell lymphoma, Lymphomatoid granulomatosis, T-cell prolymphocytic leukaemia, Splenic B-cell lymphoma/leukaemia with prominent nucleoli (formerly B-cell prolymphocytic leukaemia and hairy cell leukaemia variant), T-cell large granular lymphocytic leukaemia, Mixed-phenotype acute leukaemia, Myeloid leukaemia of Down syndrome, Burkitt leukaemia, Marginal zone lymphoma, Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome), Primary mediastinal (thymic) large B-cell lymphoma, Leukaemia (all types), Acute myeloid leukaemia, Acute lymphoblastic leukaemia, Chronic lymphocytic leukaemia, Chronic myeloid leukaemia (CML), Diffuse large B-cell lymphoma, Follicular lymphoma, Hodgkin lymphoma, Mantle cell lymphoma, Multiple myeloma, Non-Hodgkin lymphoma (all types), Myelodysplastic syndromes / neoplasms (MDS), Myeloproliferative neoplasms (PV, ET, myelofibrosis), Polycythaemia vera (PV), Essential thrombocythaemia (ET), Waldenström macroglobulinaemia, Hairy cell leukaemia, Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma), Blastic plasmacytoid dendritic cell neoplasm (BPDCN), Burkitt lymphoma, HIV-associated (AIDS-related) lymphomas, Chronic myelomonocytic leukaemia and MDS/MPN overlap neoplasms, Systemic mastocytosis, Erdheim-Chester disease, Rosai-Dorfman disease and other histiocytic neoplasms, Langerhans cell histiocytosis (LCH), Post-transplant lymphoproliferative disorder (PTLD), FLT3-mutated acute myeloid leukaemia, IDH1- and IDH2-mutated acute myeloid leukaemia, NPM1-mutated and KMT2A-rearranged acute myeloid leukaemia, Secondary and therapy-related acute myeloid leukaemia, Acute promyelocytic leukaemia, Acute myeloid leukaemia in older or unfit patients, Smouldering multiple myeloma, Newly diagnosed multiple myeloma, transplant-eligible, Newly diagnosed multiple myeloma, transplant-ineligible, Relapsed or refractory multiple myeloma, Plasma cell leukaemia, Lower-risk myelodysplastic syndromes, Higher-risk myelodysplastic syndromes, Chronic lymphocytic leukaemia, first treatment, Relapsed or refractory chronic lymphocytic leukaemia, Richter transformation of chronic lymphocytic leukaemia, Chronic myeloid leukaemia, chronic phase, Chronic myeloid leukaemia, accelerated and blast phase, Primary myelofibrosis, Standard-risk B-cell acute lymphoblastic leukaemia in children, High-risk acute lymphoblastic leukaemia in children (high-risk B-ALL and T-ALL), Philadelphia chromosome-positive acute lymphoblastic leukaemia in children (Ph-positive ALL), Philadelphia chromosome-like acute lymphoblastic leukaemia (Ph-like or BCR::ABL1-like ALL), Infant acute lymphoblastic leukaemia (KMT2A-rearranged, under one year), Relapsed and refractory acute lymphoblastic leukaemia in children, Acute myeloid leukaemia in children, Erdheim-Chester disease, Rosai-Dorfman-Destombes disease, Single-system Langerhans cell histiocytosis (bone, skin or one other organ), Multisystem Langerhans cell histiocytosis (with or without risk-organ involvement), Indolent and smouldering systemic mastocytosis, Advanced systemic mastocytosis (aggressive SM, SM with an associated haematological neoplasm, mast cell leukaemia), Early-stage classical Hodgkin lymphoma (stage I to II), Advanced-stage classical Hodgkin lymphoma (stage III to IV), Nodular lymphocyte-predominant Hodgkin lymphoma (nodular lymphocyte-predominant B-cell lymphoma), Relapsed and refractory classical Hodgkin lymphoma
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.
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.
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Query for this cancer: (TITLE:"Plasmablastic lymphoma" OR ABSTRACT:"Plasmablastic lymphoma" OR TITLE:"PBL" OR ABSTRACT:"PBL" OR TITLE:"Plasmablastic lymphoma of the oral cavity" OR ABSTRACT:"Plasmablastic lymphoma of the oral cavity" OR TITLE:"Plasmablastic lymphoma, HIV-associated" OR ABSTRACT:"Plasmablastic lymphoma, HIV-associated") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Plasmablastic lymphoma, not a curated reading list.
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.
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.
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.
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Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
A swollen painful calf, or sudden breathlessness with chest pain; venous and arterial thromboembolism is a boxed warning and blood-thinning prophylaxis is recommended.
Cumulative dose: risk rises steeply above 400-550 mg/m² (see the anthracycline calculator).
Reduce to 75% for CrCl 15-50.
Dose by creatinine clearance: 10 mg daily for CrCl 30-60, 15 mg every other day below 30, 5 mg daily on dialysis.
Start at 0.7 mg/m² in moderate or severe impairment.
See all on the product pages:BortezomibCyclophosphamideDoxorubicinEtoposideLenalidomide·Printable cards in the navigator
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