Primary effusion lymphoma
Prepared with OnCo (onco.cc/prep/primary-effusion-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 Kaposi sarcoma herpesvirusin the tumour nuclei, by immunohistochemistry for the latency-associated nuclear antigen; this is the diagnosis, Epstein-Barr virus, usually positive in people with HIV and usually negative in older people without, A plasmablastic phenotype: CD45 positive, CD20 and CD79a usually negative, CD138 and MUM1 positive, HIV status, which changes the whole of the management, Absence of the virus, which moves the diagnosis to fluid overload-associated large B-cell lymphoma and changes the outlook), 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 on the fluid), 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 Cyclophosphamide, Doxorubicin, Etoposide, 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 “There has never been a randomised trial in primary effusion lymphoma, and median overall survival in the most recent population series was 12 months”. How does that affect my plan?
- 11.I read that “The boundaries between the conditions caused by Kaposi sarcoma herpesvirus are acknowledged by the classification itself to be unclear, and individual patients overlap between them”. How does that affect my plan?
The words I may hear
- HHV-8 (KSHV) status and LANA-1 immunohistochemistry: Kaposi sarcoma, primary effusion lymphoma and one form of Castleman disease are all caused by human herpesvirus 8; a nuclear stain for its LANA-1 protein confirms the diagnosis on a biopsy, and the amount of virus in the blood tracks the dangerous inflammatory syndromes it can cause.
- 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 on the fluid: The effusion is drained and sent, fresh, for cytology, flow cytometry and immunohistochemistry. The diagnosis requires the Kaposi sarcoma herpesvirus latency-associated nuclear antigen in the tumour nuclei. A cavity lymphoma without the virus is a different disease, recognised separately in 2022, that arises in fluid overload from heart failure, kidney failure or cirrhosis and behaves better. HIV testing is part of the work-up in every case.
Biomarker results to ask for: Kaposi sarcoma herpesvirus (human herpesvirus 8) in the tumour nuclei, by immunohistochemistry for the latency-associated nuclear antigen; this is the diagnosis, Epstein-Barr virus, usually positive in people with HIV and usually negative in older people without, A plasmablastic phenotype: CD45 positive, CD20 and CD79a usually negative, CD138 and MUM1 positive, HIV status, which changes the whole of the management, Absence of the virus, which moves the diagnosis to fluid overload-associated large B-cell lymphoma and changes the outlook.
Scans and tests linked to this cancer: 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, with antiretroviral therapy started or optimised at the same time where there is HIV, because controlling the HIV is part of controlling the lymphoma. Anti-CD20 antibodies have no role: the cells do not carry CD20. There has never been a randomised trial in this entity and the regimens come from series and from the HIV-associated lymphoma literature; they sit in the treatment layer of this family and on the HIV-associated lymphoma page. Entry into a trial is a reasonable first choice rather than a last resort. (HIV-associated (AIDS-related) lymphomas, 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.