Metastatic B-cell lymphoma masquerading as infectious retinitis and vasculitis

Abstract

Abstract. Intraocular lymphoma is a rare ocular malignancy that may occur in the retina or the uvea. Retina or vitreoretinal lymphoma accounts for the majority of cases and is often secondary to diffuse large B-cell lymphoma. In the present study, a 66-year-old Caucasian male with a history of Waldenstrom's macroglobulinemia with diffuse large B-cell lymphoma, presented with blurred vision in the left eye one month following cycle 4 of an R-CHOP regimen. At the time of onset, the patient was being treated for bacterial pneumonia. Visual acuity was 20/25 in his right eye (OD) and 20/30 in the left (OS). Ophthalmologic examination showed intraretinal white infiltrates associated with hemorrhage in the superotemporal midperiphery of the retina and vitritis OS. Initial diagnostic considerations included infectious (cytomegalovirus retinitis, syphilis, toxoplasmosis, tuberculosis), inflammatory (retinal vasculitis associated with autoimmune disease or hypercoagulable states) or malignant (intraocular lymphoma) diseases. The patient did not respond to intravitreal injection of foscarnet and oral valgancyclovir. Systemic work-up and aqueous fluid biopsy were inconclusive. Diagnostic vitrectomy yielded inconclusive results and the patient continued to have progressive loss of vision. A repeat diagnostic vitrectomy with retinal and subretinal biopsy confirmed large B cells consistent with metastatic B-cell lymphoma. A concomitant PET/CT scan was performed that revealed bilateral new pulmonary nodules resulting in additional chemotherapy. Our case shows the diagnostic dilemmas in patients with systemic lymphoma and the possible role of concurrent systemic restaging in patients with ocular complaints, even when in systemic remission.

Presumed Ocular Relapse of Diffuse Large B Cell Uterine Lymphoma Presenting Only as Vitritis after Continuous and Complete Remission

“…Successful confirmatory biopsy by fine-needle aspiration or vitrectomy is a demanding procedure that requires significant skill by the ophthalmologist and also the cytopathologist [9]. Cytopathologists employ other supplementary methods, such as flow cytometry, immunohistochemistry, cytokine analyses, and molecular analysis, due to the limited amount of malignant cells that may be present in the specimen.…”

Improving the cytological diagnosis of intraocular lymphoma from vitreous fluid

“…This case also underlines the problem of patients who were thought to have suffered from infectious disease at the beginning of their clinical history. Indeed, vitroretinal lymphomas, primary or metastatic, are sometimes clinically hidden by a diagnosis of infectious retinitis or vasculitis, thus making it essential to perform a systematic vitreous sample examination, as in the case of any clinically suspected lymphoma …”

“…Indeed, vitroretinal lymphomas, primary or metastatic, are sometimes clinically hidden by a diagnosis of infectious retinitis or vasculitis, thus making it essential to perform a systematic vitreous sample examination, as in the case of any clinically suspected lymphoma. 29 Cytological analysis and ancillary studies helped us to reject a lymphoma and make a diagnosis of reactive infiltrate in 26 vitreous fluids. Uveitis may be associated with a variety of diseases, including neurological or systemic diseases.…”

Vitreoretinal lymphoma followed by systemic diffuse large B cell lymphoma

“…When systemic lymphoma results in intraocular manifestations, the involvement is typically choroidal [1, 2]. However, recent reports of vitreoretinal lymphoma in the setting of systemic lymphoma have provided insight into the clinical presentation of non-primary vitreoretinal lymphoma (VRL) [3–6]. Rapid diagnosis, work-up, and treatment of secondary VRL (SVRL) are paramount as it can commonly masquerade as primary vitreoretinal lymphoma (PVRL) as well as retinitis, uveitis, and vasculitis [4, 6].…”

“…However, recent reports of vitreoretinal lymphoma in the setting of systemic lymphoma have provided insight into the clinical presentation of non-primary vitreoretinal lymphoma (VRL) [3–6]. Rapid diagnosis, work-up, and treatment of secondary VRL (SVRL) are paramount as it can commonly masquerade as primary vitreoretinal lymphoma (PVRL) as well as retinitis, uveitis, and vasculitis [4, 6]. Here, we describe a case with a primary presentation of vitreous hemorrhage and underlying exudative retinal detachment secondary to an undiagnosed secondary vitreoretinal diffuse large B cell lymphoma (DLBCL) associated with a systemic DLBCL.…”