From the Guidelines
The management of Hodgkin lymphoma with central nervous system (CNS) involvement requires an aggressive multimodal approach, prioritizing high-dose methotrexate and systemic chemotherapy, followed by consolidation with whole-brain radiation therapy and consideration for autologous stem cell transplantation. Treatment typically consists of high-dose methotrexate (3-8 g/m²) combined with systemic chemotherapy regimens such as ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine) or escalated BEACOPP (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, prednisone) 1. This approach is often followed by consolidation with whole-brain radiation therapy (WBRT) at doses of 30-36 Gy and consideration for autologous stem cell transplantation.
Key Considerations
- CNS involvement in Hodgkin lymphoma is extremely rare (less than 0.5% of cases) and carries a poor prognosis, necessitating this intensive approach 1.
- Treatment decisions should be individualized based on the patient's age, performance status, extent of CNS involvement (parenchymal versus leptomeningeal), and presence of systemic disease.
- Close monitoring with serial MRI imaging and CSF analysis (when applicable) is essential to assess treatment response.
- Due to the rarity of this presentation, treatment is often extrapolated from approaches used for CNS involvement in non-Hodgkin lymphomas and should ideally be managed at specialized centers with experience in treating complex lymphoma cases.
Additional Treatment Options
- Intrathecal chemotherapy with methotrexate (12-15 mg), cytarabine (40-50 mg), or both may be administered for leptomeningeal disease.
- The use of novel drugs and alternating non-cross-resistant chemotherapy lines may also be considered to increase the proportion of chemosensitive patients 1.
From the Research
Management Approach for Hodgkin Lymphoma with CNS Involvement
The management of Hodgkin lymphoma (HL) with central nervous system (CNS) involvement is a complex process that requires a multidisciplinary approach.
- Initial Therapy: The initial therapy for HL patients is based on the histology of the disease, the anatomical stage, and the presence of poor prognostic features 2, 3, 4.
- Combined Modality Strategies: Patients with early-stage disease are typically treated with combined modality strategies utilizing abbreviated courses of combination chemotherapy followed by involved-field radiation therapy, while those with advanced-stage disease receive a longer course of chemotherapy often without radiation therapy 5, 2, 3, 4.
- Newer Agents: Newer agents, including brentuximab vedotin and anti-programmed death-1 (PD-1) antibodies, are now being incorporated into frontline therapy 2, 3, 4.
- High-Dose Chemotherapy and Autologous Stem Cell Transplant: High-dose chemotherapy (HDCT) followed by an autologous stem cell transplant (ASCT) is the standard of care for most patients who relapse following initial therapy 5, 2, 3, 4.
- Salvage Therapy: For patients who fail HDCT with ASCT, brentuximab vedotin, PD-1 blockade, non-myeloablative allogeneic transplant, or participation in a clinical trial should be considered 5, 2, 3, 4.
CNS Involvement
However, there is limited information available on the specific management of HL with CNS involvement in the provided studies.