Understanding R/R PCNSL
In PCNSL, progression is likely and prognosis is poor1,2

What Is Primary Central Nervous System Lymphoma (PCNSL)?

PCNSL is a rare, highly malignant non-Hodgkin lymphoma (NHL) that primarily affects people aged >60 years. PCNSL accounts for1,3-5:

2%-3% of all NHL diagnoses

4% of CNS malignancies

  • In the US, about 0.45 cases per 100,000 people are diagnosed every year6
    • An estimated 1900 people will be diagnosed with PCNSL in 2023
  • The median age at diagnosis is 65 years, and older patients5,7:
    • Frequently present with comorbidities
    • Are less likely to tolerate treatment
    • Have a higher risk of neurotoxicity
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Relapsed and Refractory Disease Is Common in Patients With PCNSL8

Many patients will either be refractory to initial treatment (ie, stable or progressive disease during or at the end of first-line treatment) or relapse after initially responding to treatment.

About 1/3

of patients may be refractory to first-line
MTX-based chemotherapy regimens

Up to 60%

of patients who respond to first-line treatment may eventually relapse

Outcomes Are Particularly Poor for Patients With R/R PCNSL

Most patients whose disease progresses will not achieve a durable second remission.2

  • Most relapses occur within the first 2 years of initial diagnosis9
7.2 Months

Median overall survival time from first disease progression to death from any cause3

Outcomes among older patients have remained largely unchanged for decades due to10:

  • Poor performance status
  • Substantial comorbidities
  • No FDA-approved treatments indicated for R/R PCNSL
What are the challenges when navigating R/R PCNSL treatment?
Current Treatment Challenges

*May include targeted agents, immunomodulatory agents, or other monotherapy/combination regimens.2

Not all drugs within the presented classes are used in the treatment of R/R PCNSL.

ASCT=autologous stem cell therapy; BTKi=Bruton tyrosine kinase inhibitor; CNS=central nervous system; FDA=Food and Drug Administration; IMiD=immunomodulatory drug; mAbs=monoclonal antibodies; MTX=methotrexate; R/R PCNSL=relapsing or refractory primary central nervous system lymphoma; WBRT=whole brain radiation therapy. 

References: 1. Ahn Y, Ahn HJ, Yoon DH, et al. Primary central nervous system lymphoma: A new prognostic model for patients with diffuse large B-cell histology. Blood Res. 2017;52(4):285-292. doi:10.5045/br.2017.52.4.285 2. Ambady P, Doolittle ND, Fox CP. Relapsed and refractory primary CNS lymphoma: treatment approaches in routine practice. Ann Lymphoma. 2021;5:23. doi:10.21037/aol-21-20 3. Tao K, Wang X, Tian X. Relapsed primary central nervous system lymphoma: Current Advances. Front Oncol. 2021;11;649789. doi.10.3389/fonc.2021.649789 4. Lv C, Wang J, Zhou M, Xu J-Y, Chen B, Wan Y. Primary central nervous system lymphoma in the United States, 1975–2017. Ther Adv Hematol. 2022;13:20406207211066166. doi:10.1177/20406207211066166 5. Schlegel U. Primary CNS lymphoma. Ther Adv Neurol Disord. 2009;2(2):93-104. doi:10.1177/1756285608101222 6. Ostrum QT, Price M, Neff C, et al. CBTRUS statistical report: primary brain and other central nervous system tumors diagnosed in the United States in 2015–2019. Neuro Oncol. 2022;24(suppl 5):v1-v95. doi: 10.1093/neuonc/noac202 7. Liu Y, Yao Q, Zhang F. Diagnosis, prognosis and treatment of primary central nervous system lymphoma in the elderly population (review). Int J Oncol. 2021;58(3):371-387. doi:10.3892/ijo.2021.5180 8. Langner-Lemercier S, Houillier C, Soussain C, et al. Primary CNS lymphoma at first relapse/progression: characteristics, management, and outcome of 256 patients from the French LOC network. Neuro Oncol. 2016;18(9):1297-1303. doi:10.1093/neuonc/now033 9. Mendez JS, Grommes C. Treatment of primary central nervous system lymphoma: from chemotherapy to small molecules. Am Soc Clin Oncol Educ Book. 2018;38:604-615. doi: 10.1200/EDBK_200829 10. Kaulen LD, Baehring JM. Treatment options for recurrent primary CNS lymphoma. Curr Treat Options Oncol. 2022;23(11):1548-1565. doi:10.1007/s11864-022-01016-5