Intraocular solitary extramedullary plasmacytoma presenting as unilateral anterior and intermediate uveitis preceded by refractory glaucoma

Tom Ayton,David Gottlieb,Svetlana Cherepanoff,Claire Hooper,William A Sewell,Sandy Smith

doi:10.1186/s12886-021-01822-9

Abstract

BackgroundSolitary extramedullary plasmacytoma (SEP) is a localised proliferation of monoclonal plasma cells involving soft tissue with no or minimal bone marrow involvement and no other systemic evidence of multiple myeloma. Intraocular involvement is exceedingly rare.Case presentationWe report a 78-year-old man who was referred with glaucoma in the right eye. He subsequently developed anterior chamber (AC) inflammation and refractory glaucoma then dense vitritis. A vitrectomy was performed with the biopsy revealing numerous plasma cells with atypical findings. In conjunction with the flow cytometry results, and a systemic work up excluding multiple myeloma, a diagnosis of SEP was made. The patient was treated with ocular external beam radiotherapy with resolution of the intraocular inflammation and control of the intraocular pressure. He remains well with no local recurrence and no development of multiple myeloma over a follow up period of 2.5 years.ConclusionsThis is the first case report of SEP presenting as intraocular inflammation without a uveal tract mass.

Highlights

Solitary extramedullary plasmacytoma (SEP) is a localised proliferation of monoclonal plasma cells involving soft tissue with no or minimal bone marrow involvement and no other systemic evidence of multiple myeloma
This is the first case report of SEP presenting as intraocular inflammation without a uveal tract mass
Uveal involvement by plasma cell neoplasms is exceedingly rare with only eight cases reported in the literature, of which only two were SEP, and one case report describing a monoclonal gammopathy of unknown significance presenting as vitritis and retinal vasculitis [4–8]

Summary

Introduction

Solitary extramedullary plasmacytoma (SEP) is a localised proliferation of monoclonal plasma cells involving soft tissue with no or minimal bone marrow involvement and no other systemic evidence of multiple myeloma. Uveal involvement by plasma cell neoplasms is exceedingly rare with only eight cases reported in the literature, of which only two were SEP, and one case report describing a monoclonal gammopathy of unknown significance presenting as vitritis and retinal vasculitis [4–8]. His BCVAs were 6/6 bilaterally, IOPs were mmHg right and mmHg left and the CDRs were unchanged.

Results

Conclusion