Plasmablastic lymphoma
Prepared with OnCo (onco.cc/prep/plasmablastic-lymphoma/). Orientation, not medical advice; your team knows your case.
My details
What I know, what is unclear, changes to discuss
Saved in this browserMy questions
11 on the sheet- 1.What is my exact diagnosis, stage, and grade, and which tests established them?
- 2.Which biomarkers have been tested on my tumour (for example 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), and what were the results?
- 3.Which subtype is my cancer, and does that change the recommended treatment?
- 4.Is germline (inherited) genetic testing recommended for me or my family?
- 5.For my situation (making the diagnosis, and why it is missed), which of the standard options do you recommend and why?
- 6.For my situation (treatment), which of the standard options do you recommend and why?
- 7.Am I a candidate for Bortezomib, Lenalidomide, Cyclophosphamide or related drugs, and what side effects should I expect?
- 8.Would a second opinion at a high-volume centre change anything, and can you help arrange it?
- 9.What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?
- 10.I read that “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”. How does that affect my plan?
- 11.I read that “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”. How does that affect my plan?
The words I may hear
- B-cell, T-cell and NK-cell lymphoma: The first thing a lymphoma report says is which kind of lymphocyte the cancer came from.
- Epstein-Barr virus (EBV) in cancer: The common glandular-fever virus, carried lifelong by most adults, which in a minority of people drives nasopharyngeal cancer, some stomach cancers and several lymphomas.
- Nodal and extranodal lymphoma: A lymphoma that starts in a lymph node is called nodal; one that starts in an organ is called extranodal.
- The two lymphoma classifications of 2022 (WHO-HAEM5 and ICC): Since 2022 there have been two reference classifications of lymphoma rather than one, published within months of each other by overlapping groups of experts.
- The lymphoma regimen alphabet: R-CHOP, pola-R-CHP, DA-EPOCH-R, ABVD, BEACOPP and the rest: Lymphoma treatment is written in acronyms, one letter per drug.
Tests and results to bring
Making the diagnosis, and why it is missed: 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.
Biomarker results to ask for: 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.
Scans and tests linked to this cancer: FDG PET, Histopathology & immunohistochemistry.
Bring copies of scan reports, pathology and blood results, and a list of every medicine and supplement.
The treatments I may be offered
- Treatment: 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. (HIV-associated (AIDS-related) lymphomas, Bortezomib, Lenalidomide, Cyclophosphamide, Doxorubicin, Etoposide, The lymphoma regimen alphabet: R-CHOP, pola-R-CHP, DA-EPOCH-R, ABVD, BEACOPP and the rest)
From the standard of care recorded for this cancer; which apply depends on your stage and biomarkers. Ask which the team recommends and why.