PHILADELPHIA — Vitreoretinal lymphoma patients with subretinal pigment epithelial infiltration had a shorter survival time while those who were younger than 55 years had a longer survival rate, a study found.
The retrospective review evaluated patient charts and obituaries of 95 vitreoretinal lymphoma patients at Wills Eye Hospital between Jan. 1, 1984, and July 30, 2018. It measured the estimated time between diagnosis and death, as well as from diagnosis of vitreoretinal lymphoma to diagnosis of central nervous system disease, which 90% of patients developed.
“Overall vitreoretinal lymphoma survival remains poor, generally ranging from 2 to 5 years,” Laura Dalvin, MD, said at the Wills Eye Conference. “We know systemic chemotherapy protocols as we have today do not improve survival. Even if we treat them prophylactically, they still go on to get CNS disease, and they tend to die in a really short course.”
The study aimed to determine risk factors for death to target patients who could benefit from new chemotherapy protocols or clinical trials.
Sub-RPE infiltration presented in 27% of patients studied, and those patients had a mean time of death of 46 months compared with 76 months for those who did not have sub-RPE infiltration.
Patients younger than 55 years lived an average of 119 months.
“We know this is a unique disease. It presents at immune-privileged sites, the eye and the brain, and it has a high prevalence of MYD88 and CD79B mutation,” Dalvin said.
Patients with a shorter time to death could be good candidates for novel therapy trials such as R-EPOCH chemotherapy protocol, which has shown to have better survival in types of CNS lymphoma, or ibrutinib, a new drug that targets the MYD88 pathway.
“I think our next avenue of exploration is to see if this drug, ibrutinib can reduce CNS lymphoma or maybe reduce the risk for death,” Carol L. Shields, MD, a co-author on the study, told Healio.com/OSN.
“We need to investigate whether more severe subtypes of lymphoma could be associated with sub-RPE infiltration, and we should consider these patients for clinical trials to both prevent CNS disease and improve survival,” Dalvin said. – by Rebecca L. Forand
Dalvin L. Survival times in patients with vitreoretinal lymphoma. Presented at: Wills Eye Conference; March 7-9, 2019; Philadelphia.
Disclosure: Dalvin reports no relevant financial disclosures.