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Cerebrospinal fluid IL-10 was normal or elevated

Intravitreal injection of methotrexate:400ug/0.1mL，Once a week\*4 weeks → Once every two weeks\*4 weeks → once a month \*10 times(16 times a year)

Systemic therapy:

Induction period:（Zanubrutinib + Rituximab，ZR） (28 days/cycle) x 6 cycles:Rituximab iv375mg/m2 D1, Zanubrutinib 160mg bid D1-D21; Maintenance treatment:Zanubrutinib 160mg bid x 2 years

Intravitreal injection of methotrexate

Yujing Qian

Meifen Zhang

This study intends to apply for the establishment of research beds, establish a Chinese PVRL research cohort, and carry out a to achieve the following research objectives:

The goal of this prospective observational study is to construct the diagnosis and treatment system for primary vitreoretinal lymphoma（PVRL）. The study is to achieve the following research objectives:

1. To establish a comprehensive diagnostic criteria for PVRL with high diagnostic efficiency and strengthen the PVRL diagnostic system;
2. To establish a standardized treatment pathway for PVRL and evaluate the efficacy and safety of treatment;
3. To screen the prognosis evaluation indicators, and to establish the follow-up process and prognosis evaluation system of PVRL；
4. To explore the pathogenesis of PVRL, specific tumor markers and drug therapeutic targets.

Construction of Diagnosis and Treatment System for Primary Vitreoretinal Lymphoma

Beijing Tongren Hospital

Southern Medical University

Peking Union Medical College Hospital

Amino Acids

Antineoplastic Agents, Immunological

Tyrosine Kinase Inhibitors

Protein Kinase Inhibitors

Tyrosine

Experimental arm will be treated with rituximab plus zanubrutinib (ZR) regimen for 6 cycles and followed by zanubrutinib maintenance for 2 years, meanwhile, intravitreal methotrexate will be given as protocol during the 1st year of treatment.

400ug intravitreal injection every week for 4 doses in the induction phase, every 2 weeks for 2 doses as consolidation, then every month for 10 doses

Rituximab 375mg/m2 intravenous infusion d1, every 21 days for 1 cycle. 6 cycles will be prescribed as protocol

160mg Bid, oral. 21-day cycle for 6 cycles in the induction phase and for 2 years in the maintenance phase

Yan Zhang, M.D.

Wei Zhang, M.M.

Hui Xu

This is a prospective single-arm phase II study, and the purpose of this study is to evaluate the efficiency of ZR regimen (rituximab \& Zanubrutinib) combined with intravitreal methotrexate and followed by Zanubrutinib maintenance in newly-diagnosed primary intraocular lymphoma. Progression-free survival (PFS) of the cohort is the primary endpoint.

All the patients will be treated with ZR regimen (Rituximab 375mg/m2 IV d1, Zanubrutinib 160mg Bid, 21 days per cycle) as induction regimen, meanwhile intravitreal methotrexate was given at a dose of 400ug for 16 doses. The response will be evaluated every 3 cycles. After 3 Cycles, patients who achieved complete remission (CR) or partial remission (PR) will receive 3 more cycles of ZR regimen. The patients with stable disease (SD) or progressed disease (PD) will withdraw from the trial and receive salvage regimens. After total 6 induction cycles, the investigators evaluate the efficiency again, the patients with CR or PR will go to Zanubrutinib maintenance for 2 years or until progression of the disease (PD), unacceptable toxicity, or patient/investigator discretion. And the patients with SD or PD will receive a salvage regimen. During following-up, surveillance ophthalmologic examination, and brain magnetic resonance imaging (MRI) scans can be performed every 3 months up to the first 2 years, followed by the doctor visit every 6 months up to 5 years or the disease relapses.

Intravitreal MTX and ZR Regimen in Newly Diagnosed PVRL

Combined Intravitreal Methotrexate and ZR (Zanubrutinib& Rituximab) Regimen Followed by Zanubrutinib Maintenance in Newly-diagnosed Primary Vitreoretinal Lymphoma

2 years progression-free survival was calculated from the date of therapy until death from lymphoma or 2-year follow up without relapsing

ORR was calculated by the proportion of patients who achieved complete remission and partial remission

Study title	NCT ID	Conditions	Interventions	Eligibility	Organization
Construction of Diagnosis and Treatment System for Primary Vitreoretinal Lymphoma	NCT05950490	Primary Vitreor...	Intravitreal in...	18 Years - 75 Years	Peking Union Medical College Hospital
Intravitreal MTX and ZR Regimen in Newly Diagnosed PVRL	NCT04899453	Primary Vitreor...	Methotrexate Rituximab Zanubrutinib	18 Years - 75 Years	Peking Union Medical College Hospital
Construction of Diagnosis and Treatment System for Primary Vitreoretinal Lymphoma	NCT05950490	Primary Vitreor...	Intravitreal in...	18 Years - 75 Years	Peking Union Medical College Hospital

Spots Global Cancer Trial Database for primary vitreoretinal lymphoma