Orelabrutinib in combination with pomalidomide for the successful treatment of primary vitreoretinal lymphoma: a case report.

BACKGROUND: Primary vitreoretinal lymphoma (PVRL) is a rare intraocular malignancy, often masquerading as chronic uveitis, which poses significant diagnostic and therapeutic challenges. CASE PRESENTATION: A 55-year-old male with 1 year of progressive bilateral vision loss was initially misdiagnosed as having bilateral uveitis and received ineffective corticosteroid therapy at an outside medical center. Subsequent diagnostic vitrectomy of the right eye confirmed bilateral PVRL. He received intravitreal methotrexate (MTX) injections for treatment but developed MTX-induced toxic keratitis and progressive cataract, necessitating discontinuation of MTX. He was transitioned to systemic therapy with anti-CD20 monoclonal antibody (discontinued due to pulmonary infection) plus oral pomalidomide and orelabrutinib, which achieved complete clearance of vitreous disease, normalization of aqueous IL-10, and sustained clinical remission without significant hematologic or cardiac toxicities. CONCLUSION: Precise diagnosis and individualized treatment regimens are required for the therapy of PVRL. Besides, the orelabrutinib-pomalidomide combination represents a promising, well-tolerated targeted and chemo free therapy strategy for PVRL.