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pcnsl
Primary CNS Lymphoma
PCNSL is ABC-DLBCL confined to CNS; MYD88 L265P ~90%, CD79B ~70%; high-dose methotrexate is the treatment backbone; ibrutinib shows ~50-60% ORR in R/R disease; 5-year OS ~30-40% with HDMTX-based therapy; vitreous IL-10 >10 pg/mL is pathognomonic.
Entry Metadata
| Field | Value |
|---|---|
| ID | pcnsl |
| Name | Primary CNS Lymphoma |
| Status | draft |
| Last reviewed | 2026-06-06 |
| Atlas | 01-human |
| Scale | 07-system |
Cross-Atlas Connections
Sources
- Bromberg JE, Issa S, Bakunina K, et al. Rituximab in patients with primary CNS lymphoma (HOVON 105/ALLG NHL 24): a randomised, open-label, phase 3 intergroup study. Lancet Oncol. 2019;20(2):216-228. · PubMed 30528440
- Grommes C, Pastore A, Palaskas N, et al. Ibrutinib unmasks critical role of Bruton tyrosine kinase in primary CNS lymphoma. Cancer Cell. 2017;31(6):833-843. · PubMed 28552327