Unusual presentation of cornea verticillata with intravitreal methotrexate in a case of primary intraocular lymphoma

A 56-year-old woman presented with floaters and diminution of vision in the right eye for 1 week. On examination, visual acuity was 20/400 in the right eye and 20/60 in the left eye. Indirect ophthalmoscopy revealed vitritis in the right eye and subretinal deposits in both eyes. Vitreous biopsy of the right eye revealed large B-cell-type primary intraocular lymphoma and the patient underwent multiple intravitreal methotrexate injections (400 μg/0.1 mL) in the right eye and systemic chemotherapy for bilateral disease. Following biweekly injections of methotrexate, her visual acuity improved considerably from 20/400 to 20/60 with resolution of vitritis. However, following eighth dose of intravitreal methotrexate, she experienced visual decline to 20/120 along with photophobia, redness and watering. Whorl-shaped opacities, limbitis and corneal haze were noted on slit-lamp examination. Intravitreal methotrexate was stopped, and the patient was started on frequent topical lubricants, loteprednol, topical folinic acid and oral folic acid. Complete resolution of corneal toxicity was observed at 3 weeks and the injections were suspended as there was no recurrence at 6 months follow-up.

Primary Intraocular Lymphoma: The Masquerade Syndrome

This chapter aims to provide a complete knowledge over the primary intraocular lymphoma (PIOL) and a correct clinical approach towards this rare condition, to avoid delays in diagnosis, which is considered the most important prognostic factor. A PIOL arises with no specific symptoms and could mimic both inflammatory and non-inflammatory ocular conditions. Also known as reticulum cell sarcoma in the past, PIOL is an ocular malignant condition, with a strong bond with primary central system lymphoma (PCNSL). This linkage is underlined by the fact that approximately 30% of the patients with PIOL have also PCNSL at presentation, while 45–90% will develop PCSNL in the following months. A correct diagnosis is currently achieved by the means of many different techniques: cytology, flow cytometry, immunohistochemistry, molecular analysis, and cytokines assay. Treatment of this condition has been completely revolutionized with the introduction of monoclonal antibodies directed against specific proteins present on the surface of lymphomatous cells.

Diagnostic retinal biopsy in the management of secondary non-CNS vitreoretinal lymphoma masquerading as viral retinitis: a case report

Background Intraocular lymphoma accounts for fewer than 1% of intraocular tumors. When the posterior segment is involved, it can be further classified as vitreoretinal or choroidal lymphoma. Vitreoretinal lymphoma (VRL) can rarely masquerade as an infectious retinitis making diagnosis and management challenging. Results A 73-year-old woman with a history of non-central nervous system (CNS) involving diffuse large B-cell lymphoma (DLBCL) was referred for worsening blurry vision—visual acuity of count figures at 2 ft—in her right eye for 8 months. Dilated fundus examination of the right eye was significant for retinal whitening and dot-blot hemorrhages, which was concerning for a viral retinitis and guided initial management. Secondary intraocular lymphoma was also considered. The retinal disease continued to progress despite intravitreal and systemic antiviral therapy, and a diagnostic vitrectomy was inconclusive. A retinal biopsy was then performed, which showed DLBCL, confirming a diagnosis of secondary VRL. Three subsequent treatments with intravitreal methotrexate led to regression of the VRL. Conclusions Our case highlights the utility of a retinal biopsy after an inconclusive diagnostic vitrectomy in a challenging scenario of VRL to establish a diagnosis and initiate successful treatment. A multidisciplinary team of providers was essential for diagnosis, comprehensive workup, medical and surgical management of the patient.

Primary Intraocular Lymphoma in a Patient with Bilateral Epstein-Barr Virus Panuveitis

Purpose. Herein, we report a case of primary intraocular lymphoma (PIOL) with the first presentation of bilateral Epstein-Barr virus- (EBV-) associated panuveitis. Case Presentation. A 69-year-old male was referred with a three-day history of blurred vision and pain and redness in his left eye following cataract surgery. Examination revealed panuveitis, vitritis, and necrotizing retinitis with retinal hemorrhage. A month later, the right eye was also involved. Polymerase chain reaction-based analysis of the vitreous sample was positive for EBV, and cytological evaluation was compatible with the diagnosis of B-cell lymphoma. A significant improvement was observed following serial intravitreal rituximab and methotrexate injections. The central nervous system and lungs were involved after 6 months, and the patient expired despite systemic chemotherapy. Conclusion. There may be an association between EBV panuveitis and primary intraocular B-cell lymphoma.

The Usefulness of Diagnostic Vitrectomy in Neoplastic and Non-Neoplastic Masquerade Syndrome: An Observational Study

Introduction: Masquerade intraocular inflammation may be considered neoplastic or non- neoplastic masquerades such as primary intraocular lymphoma, leukemia, infectious and inflammatory diseases. These pathologies require a definitive diagnosis, as the treatment modalities are different. The aim of our study was to investigate the safety and usefulness of diagnostic vitrectomy with vitreous humor flow cytometry in eyes with intraocular inflammation of unknown etiology. Methods: A retrospective observational study included 35 eyes of 29 patients with atypical intraocular inflammation unresponsive to corticosteroid therapy. In all cases diagnostic vitrectomy with flow cytometry analysis of the vitreous specimen was performed. Results: Among 35 eyes, the result of diagnostic vitrectomy analysis showed unspecific inflammatory response in 7 (20.0%) eyes, confirmed neoplastic diseases in 5 (14.3%) eyes. All of them it was intraocular lymphoma but one of the eyes with primarily diagnosed lymphoma and one of the eyes with primarily diagnosed unspecific inflammatory response in flow cytometry has been diagnosed finally as a choroidal melanoma after enucleation of the eyeball. Diagnostic vitrectomy excluded neoplastic disease in 7 eyes (20.0%). In 3 eyes (8.6%) bacterial infection, in 4 eyes (11.4%) viral infection. In 2 eyes (5.7%) we excluded bacterial infection, in 7 cases (20.0%) no conclusive results were obtained. The most common adverse event was cataract in patients (12 eyes, 34.3%). Conclusion: Diagnostic vitrectomy with flow cytometry of vitreous humor is helpful in confirming the clinical suspected diagnosis of posterior segment inflammation. Flow cytometry need to be complemented with other diagnostic test including cytopathology, especially in cases suspected of intraocular lymphoma. Flow cytometry of the vitreous humor in choroidal melanoma is not a useful diagnostic tool.

Bilateral iris mass as the first manifestation of NK cell lymphoma: A case report

Introduction: Natural killer (NK) cell lymphoma is a progressive non-Hodgkin’s lymphoma with rare prevalence. Intraocular involvement of NK cell lymphoma is very rare, with only case reports to date. Case presentation: Here, we report a case of intraocular involvement of NK cell lymphoma which was presented with bilateral uveitis, iris mass, and diffuse infiltration into the center nerve system (CNS), skin, and possibly myocardium. Conclusions: Iris mass and uveitis which is not responsive to steroids may be ocular manifestations of intraocular lymphoma, such as NK cell lymphoma. Ophthalmological and systemic examinations should be required for early diagnosis and treatment.