Immunodeficiency-associated primary CNS lymphomas: An International Primary CNS Lymphoma Collaborative Group (IPCG) Study - PubMed
6 hours ago
- #Prognostic Model
- #CNS Lymphoma
- #Immunodeficiency
- Immunodeficiency-associated primary CNS lymphoma (ID-PCNSL) is a distinct subtype with limited large-scale studies.
- An international retrospective study analyzed 308 ID-PCNSL cases from 23 sites across 7 countries.
- Common causes of immunodeficiency included immunosuppressants (41.2% for transplants, 36.7% for autoimmunity) and HIV infection (21.7%).
- All tumors were diffuse large B-cell lymphomas, with EBV detected in 79.2% of cases.
- Immune reconstitution combined with rituximab-methotrexate-based chemotherapy showed the best response rates and prolonged progression-free survival.
- Median overall survival was 54 months, with age, Karnofsky Performance Status (KPS) < 70, and EBV positivity identified as poor prognostic factors.
- A new prognostic score was developed based on age > 60, KPS < 70, and EBV positivity, stratifying survival into three risk groups (135, 29, and 3 months).
- This prognostic model outperformed existing MSKCC and IELSG models for immunocompetent PCNSL.