RARE DISEASE
Classic Hodgkin lymphoma
Classic Hodgkin lymphoma
Classic Hodgkin lymphoma
Synonyms: Classic Hodgkin disease
Synonyms: Classic Hodgkin disease
Synonyms: Classic Hodgkin disease
Drug discovery
6
drugs
With orphan designations
Overview
Classic Hodgkin lymphoma (cHL) is a B-cell malignancy characterized by Hodgkin-Reed-Sternberg cells within an inflammatory microenvironment. It exhibits bimodal age distribution and high curability (85-90% 5-year survival) with modern therapies [1][6][15]. First-line treatment combines chemotherapy (ABVD, BEACOPP) with response-adapted radiotherapy [3][5][13], while relapsed/refractory cases utilize salvage regimens, immunotherapy (PD-1 inhibitors), and stem cell transplantation [3][8][13].
Population
Bimodal incidence: peaks at 20-34 years (31.2% of cases) and >55 years [2][12]
Male predominance (M:F ≈ 1.4:1), higher incidence in high-income countries (2.8/100k vs 0.44/100k) [2][12][7]
Disparities: Hispanic patients present younger (mean 38 vs 41 years) with higher uninsured rates (15% vs 6%) [7]
Categories: rare hematological diseases, rare neoplastic diseases, rare transplant-related disorders
Research Papers
1,320 drug discovery papers related to Classic Hodgkin lymphoma, with 3 first-in-class and 12 next-in-class early-stage therapies forecasted to outperform the average preclinical success rate. Recent publications:
1,320 drug discovery papers related to Classic Hodgkin lymphoma, with 3 first-in-class and 12 next-in-class early-stage therapies forecasted to outperform the average preclinical success rate. Recent publications:
2026-07-06 | Table 1_Sufficient-dose Brentuximab vedotin improves prognosis in patients with classic Hodgkin lymphoma: a single-center real-world study in China.docx
Background Classic Hodgkin lymphoma (cHL) is highly curable for most patients with cytotoxic chemotherapy, while targeted agents such as brentuximab vedotin (BV) have been incorporated into frontline regimens. This single-center retrospective study aimed to compare the efficacy and safety of a local ABVD-like regimen and a BV-containing A-AVD-like regimen in Chinese patients with cHL. Methods A total of 243 cHL patients were enrolled, including 192 in ABVD and 51 in A-AVD. Baseline demographics, treatment outcomes, survival, and adverse events were analyzed; the median follow-up was 21.47 months. Subgroup analysis was performed including Ann Arbor stage and dose of Brentuximab Vedotin (BV). Results At baseline, the A-AVD group had a significantly higher proportion of elderly patients (≥60 years) compared to the ABVD group (25.5% vs. 9.4%, p = 0.002). First-evaluation complete response (CR: 69.3% vs. 72.5%, p = 0.549) and overall response rate (ORR: 93.8% vs. 98.0%, p = 0.312) were similar between ABVD and A-AVD. Overall progression-free survival (PFS) and overall survival (OS) showed no significant differences between the two regimens (PFS p=0.489; OS p = 0.230). The 2-year PFS rates were 71.1% for ABVD and 76.7% for A-AVD. However, subgroup analysis revealed that in patients with Ann Arbor stage III-IV, a sufficient dose of BV (≥1.15 mg/kg) significantly improved PFS compared to a reduced dose (<1.15 mg/kg) (2-year PFS 91.7% vs. 58.7%; p=0.036). Regarding safety, the A-AVD group exhibited a higher rate of peripheral neuropathy (47.1% vs. 5.2%, p < 0.001), while pulmonary toxicity events were numerically more frequent in the ABVD group. The incidences of grade 3–4 neutropenia were comparable between the two groups (17.7% in ABVD vs. 19.6% in A-AVD, p = 0.751). Conclusion This is the first real-world study of frontline A-AVD-like therapy for cHL in China. Despite a higher proportion of elderly patients in A-AVD, the regimen still achieved a 2-year PFS rate of 76.7% and showed overall efficacy and survival outcomes comparable to the ABVD-like regimen. Notably, maintaining sufficient BV dose intensity (≥1.15 mg/kg) was significantly associated with superior PFS in patients with advanced-stage (Ann Arbor III-IV) disease. While A-AVD was associated with higher rates of peripheral neuropathy, ABVD showed a trend toward a higher risk of pulmonary toxicity. These findings support careful maintenance of BV dose intensity when clinically feasible, together with proactive toxicity management. Longer follow-up and larger prospective studies are needed to validate survival outcomes and toxicity profiles in Chinese patients.
2026-07-06 | Sufficient-dose Brentuximab vedotin improves prognosis in patients with classic Hodgkin lymphoma: a single-center real-world study in China
Background Classic Hodgkin lymphoma (cHL) is highly curable for most patients with cytotoxic chemotherapy, while targeted agents such as brentuximab vedotin (BV) have been incorporated into frontline regimens. This single-center retrospective study aimed to compare the efficacy and safety of a local ABVD-like regimen and a BV-containing A-AVD-like regimen in Chinese patients with cHL. Methods A total of 243 cHL patients were enrolled, including 192 in ABVD and 51 in A-AVD. Baseline demographics, treatment outcomes, survival, and adverse events were analyzed; the median follow-up was 21.47 months. Subgroup analysis was performed including Ann Arbor stage and dose of Brentuximab Vedotin (BV). Results At baseline, the A-AVD group had a significantly higher proportion of elderly patients (≥60 years) compared to the ABVD group (25.5% vs. 9.4%, p = 0.002). First-evaluation complete response (CR: 69.3% vs. 72.5%, p = 0.549) and overall response rate (ORR: 93.8% vs. 98.0%, p = 0.312) were similar between ABVD and A-AVD. Overall progression-free survival (PFS) and overall survival (OS) showed no significant differences between the two regimens (PFS p=0.489; OS p = 0.230). The 2-year PFS rates were 71.1% for ABVD and 76.7% for A-AVD. However, subgroup analysis revealed that in patients with Ann Arbor stage III-IV, a sufficient dose of BV (≥1.15 mg/kg) significantly improved PFS compared to a reduced dose (<1.15 mg/kg) (2-year PFS 91.7% vs. 58.7%; p=0.036). Regarding safety, the A-AVD group exhibited a higher rate of peripheral neuropathy (47.1% vs. 5.2%, p < 0.001), while pulmonary toxicity events were numerically more frequent in the ABVD group. The incidences of grade 3–4 neutropenia were comparable between the two groups (17.7% in ABVD vs. 19.6% in A-AVD, p = 0.751). Conclusion This is the first real-world study of frontline A-AVD-like therapy for cHL in China. Despite a higher proportion of elderly patients in A-AVD, the regimen still achieved a 2-year PFS rate of 76.7% and showed overall efficacy and survival outcomes comparable to the ABVD-like regimen. Notably, maintaining sufficient BV dose intensity (≥1.15 mg/kg) was significantly associated with superior PFS in patients with advanced-stage (Ann Arbor III-IV) disease. While A-AVD was associated with higher rates of peripheral neuropathy, ABVD showed a trend toward a higher risk of pulmonary toxicity. These findings support careful maintenance of BV dose intensity when clinically feasible, together with proactive toxicity management. Longer follow-up and larger prospective studies are needed to validate survival outcomes and toxicity profiles in Chinese patients.
2026-07-02 | Role of radiotherapy in refractory/relapsed classical Hodgkin lymphoma in the era of targeted therapies and immunotherapy.
Relapsed or refractory classical Hodgkin lymphoma (R/R cHL) remains a complex therapeutic challenge despite major advances in systemic treatment. Salvage therapy followed by autologous stem cell transplantation (ASCT) remains the standard of care for eligible patients, but contemporary pre-transplant strategies increasingly incorporate brentuximab vedotin (BV) and, more recently, PD-1 inhibitors, which have reshaped the salvage landscape and challenged chemotherapy-only approaches. In this evolving context, radiotherapy (RT) continues to play an important role, although its indications have become more selective and individualized. Available data suggest that RT is particularly relevant for local control of residual disease, limited relapse, or metabolically active lesions, especially in the peri-transplant setting and after novel agents. Peri-transplant RT appears most useful in patients at high risk of locoregional relapse, including those with bulky disease, primary refractory lymphoma, residual PET positivity, or incomplete response before ASCT. By contrast, evidence supporting RT after or in combination with novel agents remains limited, largely derived from small, retrospective studies. Data on RT after BV are scarce but suggest excellent local control in carefully selected patients, while the combination of RT and PD-1 blockade appears especially promising, with encouraging efficacy signals from case reports, retrospective series, and recent prospective pediatric/AYA data. Modern RT techniques (IMRT/VMAT, IGRT, DIBH, and involved-site RT) enable response-adapted treatment delivery with improved normal tissue sparing. Overall, RT remains a valuable component of salvage strategies in R/R cHL in the era of BV and PD-1 inhibitors, but prospective studies are needed to better define patient selection, sequencing, target volumes, and dose prescriptions.
2026-07-06 | Table 1_Sufficient-dose Brentuximab vedotin improves prognosis in patients with classic Hodgkin lymphoma: a single-center real-world study in China.docx
Background Classic Hodgkin lymphoma (cHL) is highly curable for most patients with cytotoxic chemotherapy, while targeted agents such as brentuximab vedotin (BV) have been incorporated into frontline regimens. This single-center retrospective study aimed to compare the efficacy and safety of a local ABVD-like regimen and a BV-containing A-AVD-like regimen in Chinese patients with cHL. Methods A total of 243 cHL patients were enrolled, including 192 in ABVD and 51 in A-AVD. Baseline demographics, treatment outcomes, survival, and adverse events were analyzed; the median follow-up was 21.47 months. Subgroup analysis was performed including Ann Arbor stage and dose of Brentuximab Vedotin (BV). Results At baseline, the A-AVD group had a significantly higher proportion of elderly patients (≥60 years) compared to the ABVD group (25.5% vs. 9.4%, p = 0.002). First-evaluation complete response (CR: 69.3% vs. 72.5%, p = 0.549) and overall response rate (ORR: 93.8% vs. 98.0%, p = 0.312) were similar between ABVD and A-AVD. Overall progression-free survival (PFS) and overall survival (OS) showed no significant differences between the two regimens (PFS p=0.489; OS p = 0.230). The 2-year PFS rates were 71.1% for ABVD and 76.7% for A-AVD. However, subgroup analysis revealed that in patients with Ann Arbor stage III-IV, a sufficient dose of BV (≥1.15 mg/kg) significantly improved PFS compared to a reduced dose (<1.15 mg/kg) (2-year PFS 91.7% vs. 58.7%; p=0.036). Regarding safety, the A-AVD group exhibited a higher rate of peripheral neuropathy (47.1% vs. 5.2%, p < 0.001), while pulmonary toxicity events were numerically more frequent in the ABVD group. The incidences of grade 3–4 neutropenia were comparable between the two groups (17.7% in ABVD vs. 19.6% in A-AVD, p = 0.751). Conclusion This is the first real-world study of frontline A-AVD-like therapy for cHL in China. Despite a higher proportion of elderly patients in A-AVD, the regimen still achieved a 2-year PFS rate of 76.7% and showed overall efficacy and survival outcomes comparable to the ABVD-like regimen. Notably, maintaining sufficient BV dose intensity (≥1.15 mg/kg) was significantly associated with superior PFS in patients with advanced-stage (Ann Arbor III-IV) disease. While A-AVD was associated with higher rates of peripheral neuropathy, ABVD showed a trend toward a higher risk of pulmonary toxicity. These findings support careful maintenance of BV dose intensity when clinically feasible, together with proactive toxicity management. Longer follow-up and larger prospective studies are needed to validate survival outcomes and toxicity profiles in Chinese patients.
2026-07-06 | Sufficient-dose Brentuximab vedotin improves prognosis in patients with classic Hodgkin lymphoma: a single-center real-world study in China
Background Classic Hodgkin lymphoma (cHL) is highly curable for most patients with cytotoxic chemotherapy, while targeted agents such as brentuximab vedotin (BV) have been incorporated into frontline regimens. This single-center retrospective study aimed to compare the efficacy and safety of a local ABVD-like regimen and a BV-containing A-AVD-like regimen in Chinese patients with cHL. Methods A total of 243 cHL patients were enrolled, including 192 in ABVD and 51 in A-AVD. Baseline demographics, treatment outcomes, survival, and adverse events were analyzed; the median follow-up was 21.47 months. Subgroup analysis was performed including Ann Arbor stage and dose of Brentuximab Vedotin (BV). Results At baseline, the A-AVD group had a significantly higher proportion of elderly patients (≥60 years) compared to the ABVD group (25.5% vs. 9.4%, p = 0.002). First-evaluation complete response (CR: 69.3% vs. 72.5%, p = 0.549) and overall response rate (ORR: 93.8% vs. 98.0%, p = 0.312) were similar between ABVD and A-AVD. Overall progression-free survival (PFS) and overall survival (OS) showed no significant differences between the two regimens (PFS p=0.489; OS p = 0.230). The 2-year PFS rates were 71.1% for ABVD and 76.7% for A-AVD. However, subgroup analysis revealed that in patients with Ann Arbor stage III-IV, a sufficient dose of BV (≥1.15 mg/kg) significantly improved PFS compared to a reduced dose (<1.15 mg/kg) (2-year PFS 91.7% vs. 58.7%; p=0.036). Regarding safety, the A-AVD group exhibited a higher rate of peripheral neuropathy (47.1% vs. 5.2%, p < 0.001), while pulmonary toxicity events were numerically more frequent in the ABVD group. The incidences of grade 3–4 neutropenia were comparable between the two groups (17.7% in ABVD vs. 19.6% in A-AVD, p = 0.751). Conclusion This is the first real-world study of frontline A-AVD-like therapy for cHL in China. Despite a higher proportion of elderly patients in A-AVD, the regimen still achieved a 2-year PFS rate of 76.7% and showed overall efficacy and survival outcomes comparable to the ABVD-like regimen. Notably, maintaining sufficient BV dose intensity (≥1.15 mg/kg) was significantly associated with superior PFS in patients with advanced-stage (Ann Arbor III-IV) disease. While A-AVD was associated with higher rates of peripheral neuropathy, ABVD showed a trend toward a higher risk of pulmonary toxicity. These findings support careful maintenance of BV dose intensity when clinically feasible, together with proactive toxicity management. Longer follow-up and larger prospective studies are needed to validate survival outcomes and toxicity profiles in Chinese patients.
2026-07-02 | Role of radiotherapy in refractory/relapsed classical Hodgkin lymphoma in the era of targeted therapies and immunotherapy.
Relapsed or refractory classical Hodgkin lymphoma (R/R cHL) remains a complex therapeutic challenge despite major advances in systemic treatment. Salvage therapy followed by autologous stem cell transplantation (ASCT) remains the standard of care for eligible patients, but contemporary pre-transplant strategies increasingly incorporate brentuximab vedotin (BV) and, more recently, PD-1 inhibitors, which have reshaped the salvage landscape and challenged chemotherapy-only approaches. In this evolving context, radiotherapy (RT) continues to play an important role, although its indications have become more selective and individualized. Available data suggest that RT is particularly relevant for local control of residual disease, limited relapse, or metabolically active lesions, especially in the peri-transplant setting and after novel agents. Peri-transplant RT appears most useful in patients at high risk of locoregional relapse, including those with bulky disease, primary refractory lymphoma, residual PET positivity, or incomplete response before ASCT. By contrast, evidence supporting RT after or in combination with novel agents remains limited, largely derived from small, retrospective studies. Data on RT after BV are scarce but suggest excellent local control in carefully selected patients, while the combination of RT and PD-1 blockade appears especially promising, with encouraging efficacy signals from case reports, retrospective series, and recent prospective pediatric/AYA data. Modern RT techniques (IMRT/VMAT, IGRT, DIBH, and involved-site RT) enable response-adapted treatment delivery with improved normal tissue sparing. Overall, RT remains a valuable component of salvage strategies in R/R cHL in the era of BV and PD-1 inhibitors, but prospective studies are needed to better define patient selection, sequencing, target volumes, and dose prescriptions.
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Drug Discovery Landscape
6 orphan drug designations for Classic Hodgkin lymphoma.
6 orphan drug designations for Classic Hodgkin lymphoma.
Drug | Therapy type | Regulator | Orphan designation | Approval | Sponsor |
|---|---|---|---|---|---|
Panobinostat [Faridak] | small molecules | EMA | 2010-02-02 | — | Novartis Europharm Limited |
Recombinant antibody construct against human CD30 and CD16A | antibodies | EMA | 2009-10-09 | — | Affimed GmbH |
Allogeneic umbilical cord blood cells-derived CD133+ cells ex vivo expanded [StemEX] | cell therapies | EMA | 2009-07-24 | — | Regulatory Resources Group Limited |
Brentuximab vedotin [Adcetris] | antibodies | EMA | 2009-01-15 | — | Takeda Pharma A/S |
Mocetinostat [MGDC0103] | small molecules | EMA | 2007-09-14 | — | ICON Clinical Research Limited |
Human IgG4 monoclonal antibody against HLA-DR | antibodies | EMA | 2005-06-16 | — | [INACTIVE] GPC Biotech AG |
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