Nodular lymphocyte-predominant Hodgkin lymphoma (nodular lymphocyte-predominant B-cell lymphoma)
Prepared with OnCo (onco.cc/prep/nodular-lymphocyte-predominant-hodgkin-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
19 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 CD20-positive, CD30- and CD15-negative LP cells with OCT2 and PAX5 expression, Fan growth patternon the biopsy, Absence of EBV, Stage and number of nodal sites, Lactate dehydrogenase and B symptoms), 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 (diagnosis), which of the standard options do you recommend and why?
- 6.For my situation (stage ia without risk factors), which of the standard options do you recommend and why?
- 7.For my situation (stage ib to iv), which of the standard options do you recommend and why?
- 8.Am I a candidate for Rituximab, Doxorubicin, Cyclophosphamide or related drugs, and what side effects should I expect?
- 9.For my situation (relapse), which of the standard options do you recommend and why?
- 10.Am I a candidate for Rituximab, and what side effects should I expect?
- 11.For my situation (transformation), which of the standard options do you recommend and why?
- 12.Am I a candidate for Rituximab, and what side effects should I expect?
- 13.For my situation (nodular lymphocyte-predominant hodgkin lymphoma: a different disease that keeps the name), which of the standard options do you recommend and why?
- 14.Am I a candidate for Rituximab, Bendamustine, Doxorubicin or related drugs, and what side effects should I expect?
- 15.Are there clinical trials I could join, for example of Rituximab?
- 16.Would a second opinion at a high-volume centre change anything, and can you help arrange it?
- 17.What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?
- 18.I read that “No randomised trial has compared ABVD with rituximab-based chemotherapy”. How does that affect my plan?
- 19.I read that “Variant growth patterns are hard to reproduce between pathologists”. How does that affect my plan?
The words I may hear
- ABVD, BEACOPP and BrECADD (Hodgkin lymphoma regimens): The chemotherapy recipes that cure most Hodgkin lymphoma: ABVD (four drugs, the long-standing standard), the more intensive German BEACOPP, and newer versions that replace bleomycin with brentuximab vedotin (A+AVD) or add nivolumab (N-AVD).
- After Hodgkin lymphoma: the late effects, and the screening that follows them: Most people treated for Hodgkin lymphoma are cured, and the long follow-up cohorts show that the treatment leaves a raised risk of heart disease, of a second cancer and of an underactive thyroid for decades.
- Late effects of Hodgkin lymphoma treatment, and the follow-up that answers them: Hodgkin lymphoma is usually cured, and the problems that follow arrive twenty and thirty years later: heart disease, an underactive thyroid, and second cancers, especially breast cancer in women irradiated to the chest when young.
- Lymphoma (tissue type): Cancer of lymphocytes, the white blood cells of the immune system, usually growing as masses in lymph nodes, spleen or other organs.
- Watch and wait in follicular lymphoma: being told you have cancer and that nobody will treat it: For a slow-growing lymphoma that is not causing problems, treating straight away has not been shown to help people live longer, so the usual plan is regular checks and treatment later.
- Secondary malignancy (therapy-related cancer): A new, different cancer caused by the treatment of the first one: leukaemia after alkylating chemotherapy or PARP inhibitors, solid tumours in irradiated tissue decades later, and rarely T-cell lymphoma after CAR-T.
- Watchful waiting, and how it differs from active surveillance: Two things that sound the same and are not.
- Watch and wait in lymphoma: when the right treatment is none yet: For slow-growing lymphomas that are not causing symptoms, treating straight away does not help people live longer.
- 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.
- Lugano classification / Ann Arbor staging: The Lugano classification is the lymphoma staging system: stage I to IV by how many lymph node regions and organs are involved, with PET-based response criteria.
Tests and results to bring
Diagnosis: Excisional node biopsy with expert haematopathology review to distinguish from classical Hodgkin lymphoma and T-cell/histiocyte-rich large B-cell lymphoma; FDG-PET/CT staging.
Biomarker results to ask for: CD20-positive, CD30- and CD15-negative LP cells with OCT2 and PAX5 expression, Fan growth pattern (A to F) on the biopsy, Absence of EBV, Stage and number of nodal sites, Lactate dehydrogenase and B symptoms (transformation suspicion), FDG-PET (staging; avid).
Scans and tests linked to this cancer: Active surveillance, FDG PET, Histopathology & immunohistochemistry, Multidisciplinary tumour boards.
Bring copies of scan reports, pathology and blood results, and a list of every medicine and supplement.
The treatments I may be offered
- Stage IA without risk factors: Involved-site radiotherapy alone (30 Gy); in children, complete excision followed by observation. (IMRT / IGRT (modern external beam), Active surveillance, Children's Oncology Group (COG))
- Stage IB to IV: ABVD or rituximab-containing chemotherapy (R-CHOP, R-CVP, R-ABVD) with or without involved-site radiotherapy; rituximab alone for frail patients. (Rituximab, R-CHOP (lymphoma chemoimmunotherapy), ABVD, BEACOPP and BrECADD (Hodgkin lymphoma regimens), Doxorubicin, Cyclophosphamide, Vincristine, IMRT / IGRT (modern external beam))
- Transformation: Treat as diffuse large B-cell lymphoma with R-CHOP. (R-CHOP (lymphoma chemoimmunotherapy), Rituximab)
- Nodular lymphocyte-predominant Hodgkin lymphoma: a different disease that keeps the name: The malignant cell expresses CD20 and not CD30 or CD15, which is the opposite of classical Hodgkin lymphoma and the reason rituximab works and brentuximab vedotin does not. The WHO fifth edition renames it nodular lymphocyte-predominant B-cell lymphoma, which is a better description. It is indolent, affects men more than women, and relapses late. Stage IA disease without risk factors is treated with involved-site radiotherapy alone, typically 30 Gy, and a substantial proportion never relapse. Complete surgical excision of a single node followed by observation is used in children and in selected adults. More advanced disease is treated with rituximab-containing systemic treatment: R-CHOP, R-ABVD or bendamustine with rituximab, with or without radiotherapy to a residual site. A retrospective population series of 23 patients treated with bendamustine and rituximab in Alberta reported a response rate of 100 per cent, complete response in 78 per cent, and four-year progression-free survival of 83 per cent and overall survival of 87 per cent, which is the kind of evidence this uncommon disease has. The long-term data make the central point about how gently it should be treated. Across 471 patients in the German Hodgkin Study Group HD7 to HD15 trials, ten-year progression-free survival was 75.5 per cent and overall survival 92.1 per cent, but second malignancies occurred in 10.2 per cent, and of 43 deaths only 10 were from the lymphoma against 20 from second cancers and 13 from possibly treatment-related conditions. Over-treatment, not the lymphoma, is the main threat to life here. Transformation to a T-cell/histiocyte-rich large B-cell lymphoma occurs in a minority and is treated as aggressive lymphoma. (Long-term follow-up of nodular lymphocyte-predominant Hodgkin lymphoma treated in the GHSG HD7 to HD15 trials, Rituximab, Bendamustine, R-CHOP (lymphoma chemoimmunotherapy), Doxorubicin, Cyclophosphamide, Vincristine, Prednisone, IMRT / IGRT (modern external beam), Radiotherapy in lymphoma: involved-site fields, 24 Gy, 4 Gy and total skin electron therapy, Watch and wait in lymphoma: when the right treatment is none yet, Late effects of Hodgkin lymphoma treatment, and the follow-up that answers them, Secondary malignancy (therapy-related cancer))
- Relapse: Rebiopsy to exclude transformation; rituximab alone or with chemotherapy, radiotherapy for localised relapse; autologous transplantation only for early or repeated relapse. (Rituximab, Autologous stem cell transplant (high-dose therapy), IMRT / IGRT (modern external beam))
From the standard of care recorded for this cancer; which apply depends on your stage and biomarkers. Ask which the team recommends and why.