From the Research
The prognosis of Hodgkin lymphoma with central nervous system (CNS) involvement is generally poor, with median survival typically ranging from 10-46 months after diagnosis of CNS disease. CNS involvement in Hodgkin lymphoma is extremely rare, occurring in less than 0.5% of cases, and usually represents advanced disease 1. Patients with isolated CNS relapse tend to have better outcomes than those with concurrent systemic disease.
Treatment Approach
Treatment typically involves a multimodal approach including:
- High-dose methotrexate (3-8 g/m² every 2-3 weeks)
- Whole brain radiation therapy (30-40 Gy)
- Systemic chemotherapy regimens such as BEACOPP (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, prednisone) or ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine) 2, 3 Autologous stem cell transplantation may be considered for eligible patients who achieve remission. The poor prognosis is attributed to the blood-brain barrier limiting drug penetration, the aggressive nature of CNS disease, and patients often being heavily pretreated with compromised performance status.
Recent Advances
Recent studies have explored the use of novel agents, including brentuximab vedotin and PD-1 blockade, in the treatment of Hodgkin lymphoma 4, 5. These agents have shown promise in improving outcomes for patients with relapsed or refractory disease, and may potentially be incorporated into frontline treatment regimens. However, the optimal approach for patients with CNS involvement remains unclear, and further research is needed to determine the best course of treatment.
Key Considerations
Early diagnosis through MRI imaging and CSF analysis is crucial, as prompt initiation of CNS-directed therapy offers the best chance for improved outcomes. Patients with CNS involvement should be managed by a multidisciplinary team, including neurologists, radiation oncologists, and medical oncologists, to ensure comprehensive care.