An international real‐world study of primary vitreoretinal lymphoma from the Australasian lymphoma alliance and collaborators
Primary vitreoretinal lymphoma (PVRL) is a high-grade extranodal non-Hodgkin lymphoma, with limited prospective data to inform practice. High rates of central nervous system (CNS) relapse contribute to its poor prognosis. This international multicentre retrospective cohort study aimed to characterise real-world contemporary practice and outcomes in PVRL (2010-2022). Sixty patients were included from 11 centres across Australia, Singapore, Canada and the United Kingdom. Most patients had systemic therapy included in their initial management (63%) either alone or in combination with local therapies; 13% had upfront autologous stem cell transplantation (ASCT). The overall response rate was 78%. With a median follow-up of 68 months, the median progression-free survival (PFS) was 25 months, with a median overall survival (OS) of 73 months. Neither incorporation of systemic therapy into initial treatment nor upfront ASCT demonstrated a statistically significant impact on PFS or OS. The 5-year cumulative incidence of CNS relapse was 33%, with front-line systemic therapy being the only predictive factor for CNS relapse in a multivariate model, hazard ratio of 0.30 (95% CI 0.09-0.98, p = 0.05). Concerning heterogeneity in real-world approaches to diagnosis, staging and management approaches were identified. Further international collaborative efforts are required to address the unmet need in this rare entity.
Authors
- Limei Michelle Poon (ORCID: https://orcid.org/0000-0002-8854-9414)
- Pam McKay (ORCID: https://orcid.org/0000-0002-3959-9730)
- Katharine L. Lewis (ORCID: https://orcid.org/0000-0003-0549-5877)
- Mandeep S. Sagoo (ORCID: https://orcid.org/0000-0003-1530-3824)
- Anca A. Prica (ORCID: https://orcid.org/0000-0001-5621-9261)
- Lucy Zhang (ORCID: https://orcid.org/0000-0002-8609-767X)
- Adam Suleman (ORCID: https://orcid.org/0000-0002-2585-1982)
- Edward Pringle (ORCID: https://orcid.org/0000-0002-3431-9998)
- Catherine Tang (ORCID: https://orcid.org/0000-0002-0223-8341)
- Jeffery Smith
- Svetlana Cherepanoff (ORCID: https://orcid.org/0000-0003-4697-3820)
- Chan Yoon Cheah (ORCID: https://orcid.org/0000-0001-7988-1565)
- Nada Hamad (ORCID: https://orcid.org/0000-0001-7929-1450)
- Shireen Kassam (ORCID: https://orcid.org/0000-0002-6173-3326)
- Luke Coyle
- Daire Quinn
- Catriona Downie
- William D. Hann
Institutions
- Moorfields Eye Hospital NHS Foundation Trust (GB)
- The University of Western Australia (AU)
- University of Toronto (CA)
- Barts Health NHS Trust (GB)
- Royal North Shore Hospital (AU)
- Beatson West of Scotland Cancer Centre (GB)
- Gosford Hospital (AU)
- Sir Charles Gairdner Hospital (AU)
- Princess Margaret Cancer Centre (CA)
- Clatterbridge Cancer Centre NHS Foundation Trust (GB)
- St Vincent's Hospital Melbourne (AU)
- St Vincent's Hospital Sydney (AU)
- National University Cancer Institute, Singapore (SG)
- King's College Hospital NHS Foundation Trust (GB)
- The Kinghorn Cancer Centre (AU)
- Linear Clinical Research (AU)
- University College London (GB)
- University of Newcastle Australia (AU)
Publication Details
- Journal
- British Journal of Haematology
- Published
- 2025-05-06
- DOI
- https://doi.org/10.1111/bjh.20115
- Citations
- 1
- Primary Topic
- CNS Lymphoma Diagnosis and Treatment
- Type
- article
- Field-Weighted Citation Impact
- 1.39
Funders
- Bristol-Myers Squibb
- AstraZeneca
- Gilead Sciences
- Celgene