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9 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
Nodular lymphocyte-predominant Hodgkin lymphoma is the rare, slow-growing cousin of classical Hodgkin lymphoma, so different in its CD20-bearing cells that the WHO renamed it a B-cell lymphoma in 2022. Early disease is usually cured with radiotherapy alone or surgery in children, advanced disease with rituximab-based chemotherapy, and patients are followed for life because it can return late.
Nodular lymphocyte-predominant Hodgkin lymphoma was separated from classical Hodgkin lymphoma in 1994 because its tumour cells, the 'popcorn' or LP cells, are CD20-positive, CD30- and CD15-negative B cells that keep their B-cell programme, sit in nodules of small B lymphocytes and follicular T-helper cells, and lack EBV; the 2022 WHO classification took the logic to its end and renamed the disease nodular lymphocyte-predominant B-cell lymphoma, while the International Consensus Classification kept the older name. It presents with a single group of peripheral nodes (neck, axilla or groin) in a young man, rarely in the mediastinum, and stays indolent for years; but a proportion of cases show T-cell-rich or diffuse growth patterns (Fan patterns C to F) that behave more aggressively and blur into T-cell/histiocyte-rich large B-cell lymphoma, into which the disease transforms in a minority over the following decades.
Treatment is gentler than for classical disease. Stage IA disease without risk factors is cured in most patients by involved-site radiotherapy alone (30 Gy), and children with a completely excised single node can be watched without further treatment, as Children's Oncology Group and EuroNet studies showed. Stage II to IV disease is treated with chemotherapy, usually ABVD or, because the cells are CD20-positive, R-CHOP or R-CVP with rituximab, which some centres prefer for its lower risk of transformation; single-agent rituximab produces responses in most patients but they are not durable. Relapse is common but slow, and relapsed disease is treated with rituximab alone or with chemotherapy, radiotherapy or, rarely, autologous transplantation; transformation is treated as diffuse large B-cell lymphoma. Survival is excellent and most deaths in older series were from treatment or second cancers, which is the reason to treat as little as possible. Because the disease is rare and heterogeneous, trials are small, and the current questions are whether rituximab-based regimens should replace ABVD in advanced disease and how to identify the variant patterns that need more.
| Setting | Approach | Guideline |
|---|---|---|
| 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. | not mapped |
| Stage IA without risk factors | Involved-site radiotherapy alone (30 Gy); in children, complete excision followed by observation. | not mapped |
| 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. | not mapped |
| Relapse | Rebiopsy to exclude transformation; rituximab alone or with chemotherapy, radiotherapy for localised relapse; autologous transplantation only for early or repeated relapse. | not mapped |
| Transformation | Treat as diffuse large B-cell lymphoma with R-CHOP. | not mapped |
| 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. | not mapped |