Primary Central Nervous System Lymphoma

Primary Central Nervous System Lymphoma (PCNSL) is a rare and aggressive non-Hodgkin lymphoma that is confined to the brain, spine, eyes, and meninges.^1,2 In PCNSL, acquired mutations lead to constitutive activation of the BCR-TLR signaling pathway which causes excessive proliferation and survival of B-cells.³

Despite high overall response rates to the guideline-recommended first-line treatment, most patients with PCNSL have refractory disease or experience disease recurrence.⁴ Up to 30% of patients have disease that is refractory to first-line treatment and up to 45% of patients who respond to first-line treatment experience disease recurrence after < 5 years after diagnosis.^4-6Currently, there are no approved treatments for patients with relapsed or refractory PCNSL.

PCNSL Materials

Phase III CTFS

References

1) Hoffman S, et al. Neuro-Oncol. 2006;8:27-37.
2) Mendez JS, et al. Neuro Oncol. 2018;20(5):687-694.
3) Wu J, et al. Ther Adv Hematol. 2024;15:20406207241259010.
4) Houillier C, et al. Neurology. 2020;94(10):e1027-e1039.
5) Tringale KR, et al. Neuro Oncol. 2024;26(11):2061-2073.
6) Kim JE, et al. Korean J Hematol. 2012;47(1):60-66.