T-cell/histiocyte-rich large B-cell lymphoma
Prepared with OnCo (onco.cc/prep/t-cell-histiocyte-rich-large-b-cell-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
10 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 Scattered large B cells, fewer than about one in ten of the cells present, in a background of small T cells and histiocytes, The large cells express CD20 and BCL6; nuclear BCL6 was positive in 26 of 29 cases in one series, A background rich in CD8-positive T cells and CD68-positive histiocytes, which is part of the diagnosis rather than incidental, Absence of the nodular meshworks of follicular dendritic cells and of the small B cells that mark nodular lymphocyte predominant Hodgkin lymphoma, Epstein-Barr virus, which is characteristically negative), and what were the results?
- 3.Is germline (inherited) genetic testing recommended for me or my family?
- 4.For my situation (making the diagnosis, and the two ways it goes wrong), which of the standard options do you recommend and why?
- 5.For my situation (treatment), which of the standard options do you recommend and why?
- 6.Am I a candidate for Rituximab, and what side effects should I expect?
- 7.Would a second opinion at a high-volume centre change anything, and can you help arrange it?
- 8.What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?
- 9.I read that “The line between this disease and the diffuse pattern of nodular lymphocyte predominant Hodgkin lymphoma cannot always be drawn, and the two are treated differently”. How does that affect my plan?
- 10.I read that “The cancer cells of the two conditions are not distinguishable by gene expression. What differs is the immune cells around them, and nobody knows why the same tumour cell produces an indolent disease in one person and an aggressive one in another”. How does that affect my plan?
The words I may hear
- International Prognostic Index (IPI): A five-point score (age, stage, performance status, LDH, extranodal sites) that predicts how risky a lymphoma is before treatment.
- B-cell, T-cell and NK-cell lymphoma: The first thing a lymphoma report says is which kind of lymphocyte the cancer came from.
- R-CHOP (lymphoma chemoimmunotherapy): R-CHOP is the standard first treatment for diffuse large B-cell lymphoma: rituximab (an antibody against CD20) plus four chemotherapy drugs (cyclophosphamide, doxorubicin, vincristine, prednisone), given every three weeks for six cycles with curative intent.
- Indolent and aggressive lymphoma: Lymphomas are split by how fast they grow, and the split decides what happens next.
- 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 the two ways it goes wrong: Both errors are common and they point in opposite directions. Called inflammatory, the lymphoma is missed, because the tumour cells are a small minority and the tissue looks reactive. Called nodular lymphocyte predominant Hodgkin lymphoma, an aggressive disease is treated as an indolent one. WHO-HAEM5 states that a clear distinction from the diffuse pattern of that disease may not be possible in some cases, and that small biopsies are the hardest. A generous biopsy, read by a haematopathologist, with the surrounding cells examined as carefully as the tumour cells, is the answer the classification gives.
Biomarker results to ask for: Scattered large B cells, fewer than about one in ten of the cells present, in a background of small T cells and histiocytes, The large cells express CD20 and BCL6; nuclear BCL6 was positive in 26 of 29 cases in one series, A background rich in CD8-positive T cells and CD68-positive histiocytes, which is part of the diagnosis rather than incidental, Absence of the nodular meshworks of follicular dendritic cells and of the small B cells that mark nodular lymphocyte predominant Hodgkin lymphoma, Epstein-Barr virus, which is characteristically negative.
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: Treated as diffuse large B-cell lymphoma, with rituximab-containing immunochemotherapy, which is appropriate for a disease that carries CD20 and behaves aggressively. The staging usually finds advanced disease with the liver, spleen or bone marrow involved. No randomised trial has been confined to this entity; the regimens, the cycles and the evidence are on the diffuse large B-cell lymphoma page and in the treatment layer of this family. (Diffuse large B-cell lymphoma, Rituximab, R-CHOP (lymphoma chemoimmunotherapy), 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.