Primary Vitreous and retinal lymphoma (PVRL): Risk factor and prognosis

Abstract

AbstractPrimary Vitreous and retinal lymphoma (PVRL): risk factor and prognosis Pr cassoux PVRL also called primary intra ocular lymphoma (PIOL) is a subset of primary cerebral lymphoma. This lymphoma is part of a heterogeneous group of non‐Hodgkin lymphoma poorly understood and widely incurable. The vast majority of patients are affected by central nervous system diffuse large B cell lymphoma (DLBCL. During the past three decades, there have been consistent reports of an increase in the incidence of NHL worldwide. In the United States, the age‐adjusted incidence rate has almost doubled since the 1970s, from 11.07 per 100 000 in 1975 to 20.20 per 100 000 in 2008 for NHL The etiology of NHL is poorly understood, with the only established risk factors being infection and immune dysregulation. In immunosuppressed patients PVRL is generally associated with EBV infection which is not the case in their immunocompetent counterpart. There is no proofs of the role of environment and exposition to chemical agents had an influence on DLBCL incidence (Interlymph data). Prognosis of PVRL is functional and linked to local progression with a risk of blindness. The poor survival is due to the risk of CNS involvement during evolution. Between 60% and 85% of patients with initially isolated PVRL will develop CNS localization within 29 months of diagnosis PVRL. Until recently, primary central nervous system lymphoma (PCNSL) was associated with a uniformly dismal prognosis. It is now reasonable to anticipate long‐term survival and possibly cure for a significant proportion of patients diagnosed with PCNSL. Poor prognosis is linked to age, performance status and associated comorbidities. A significant number of patients get prolonged survival with intensive chemotherapy plus consolidation.