Abstract

(1) Purpose: Primary vitreoretinal lymphoma (PVRL) is associated with poor prognosis because most of the patients with PVRL develop central nerve system lymphoma. The prognostic biomarker of PVRL is largely unknown. Cluster analysis has been used to identify phenotypic groups within various diseases. In this study, we aimed to describe clinical features of patients with PVRL grouped by clustering analysis and to identify biomarkers for predicting survival prognosis in patients with PVRL. (2) Materials and Methods: Forty patients with PVRL were divided into two groups by clustering analysis using clinical data. Clinical features of the two groups were compared. (3) Result: Clustering analysis classified patients into groups A and B. The survival rate during the follow-up period was significantly lower in group B than in group A (p = 0.03). Serum IgG, serum IgA, vitreous IL-10 and vitreous IL-10 to IL-6 ratio were significantly different between groups A and B (p = 0.03, 0.005, 0.008 and 0.03, respectively). Receiver operating characteristic (ROC) curves generated for the four variables indicated that serum IgA was most suitable for the prediction of prognosis. Patients with serum IgA below 184 mg/dL obtained from the ROC curve had a lower three-year survival rate (p = 0.03) and more episodes of recurrence of lymphoma (3.2 times versus 1.8 times, p = 0.02) compared with patients with serum IgA above 184 mg/dL. (4) Conclusion: The survival rate was significantly different in PVRL patients classified into two groups by clustering analysis. Patients with lower serum IgA had more recurrences and poorer survival than patients with higher IgA.

Highlights

  • Primary vitreoretinal lymphoma (PVRL), previously known as primary intraocular lymphoma (PIOL), is a form of non-Hodgkin’s lymphoma of the diffuse large B cell type (DLBCL) and constitutes approximately less than 1% of all intraocular tumors [1]

  • Patients with serum immunoglobulin A (IgA) below 184 mg/dL obtained from the Receiver operating characteristic (ROC) curve had a lower three-year survival rate (p = 0.03) and more episodes of recurrence of lymphoma (3.2 times versus 1.8 times, p = 0.02) compared with patients with serum IgA above 184 mg/dL. (4) Conclusion: The survival rate was significantly different in PVRL patients classified into two groups by clustering analysis

  • PVRL is associated with poor prognosis because most of the patients with PVRL develop central nerve system lymphoma (CNSL) within a few years after onset of PVRL

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Summary

Introduction

Primary vitreoretinal lymphoma (PVRL), previously known as primary intraocular lymphoma (PIOL), is a form of non-Hodgkin’s lymphoma of the diffuse large B cell type (DLBCL) and constitutes approximately less than 1% of all intraocular tumors [1]. PVRL is associated with poor prognosis because most of the patients with PVRL develop central nerve system lymphoma (CNSL) within a few years after onset of PVRL. Misdiagnosis of PIOL at onset increases the risk of missed or delayed diagnosis of CNSL [6,7,8,9]. For this reason, PVRL is diagnosed for the first time after onset of CNSL in some patients. Our previous multicenter study in Japan which analyzed the clinical course of PIOL found that 60% of the patients had both PIOL and CNSL lesions and that the five-year survival rate was 61% [3]

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