Secondary Vitreoretinal Lymphoma With Spontaneous Regression
Kongwattananon, W., Kumar, A., Simard, J., Wiley, H. E., Sen, H. N., & Kodati, S. (2022). Secondary vitreoretinal lymphoma with spontaneous regression. American journal of ophthalmology case reports, 25, 101357. https://doi.org/10.1016/j.ajoc.2022.101357
View Original Source →Abstract
Purpose To report a patient with vitreoretinal lymphoma (VRL) secondary to systemic diffuse large B-cell lymphoma, who had two episodes of spontaneous regression. Observations An 80-year-old Nicaraguan male with a history of treated systemic diffuse large B-cell lymphoma presented with decreased vision in his right eye over one year. The patient was found to have subretinal lesions and moderate vitreous opacities in his right eye. Cytological analysis of vitreous confirmed B-cell lymphoma. Following his systemic work-up, spontaneous clinical improvement was noted. There were no vitreoretinal or systemic lymphoma recurrences during one year of follow-up until the patient had new onset decreased vision in the left eye. He was presumed to have a recurrence of VRL supported by optical coherence tomography findings. Repeat systemic workup was negative for reoccurrence and the ocular lesions resolved spontaneously over 4 weeks. Conclusions Spontaneous regression of intraocular lymphoma can rarely occur. Multimodal imaging has an essential role in diagnosing and monitoring recurrence of this disease.
Case Details
Disease Location
Eye
Personal Characteristics
80-year-old nicaraguan male. Diagnosed with diffuse-large b cell lymphoma (dlbcl) stage iib 3 years earlier, he completed six cycles of the r-chop (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy regimen and achieved remission. History of non-clearing vitreous hemorrhage of unclear etiology in the right eye for which pars plana vitrectomy was performed.
Clinical Characteristics
Presented with a history of painless decreased vision in his right eye. On initial ophthalmological examination, best-corrected visual acuity (bcva) was hand movements (hm) in the right eye and 20/40 in the left eye. A right relative afferent pupillary defect was present. Slit-lamp examination revealed 1+ anterior chamber cell, 1+ vitreous cell, and trace vitreous haze in the right eye. His right fundus exam was notable for diffuse intraretinal and subretinal infiltration, affecting predominantly the optic disc and peripapillary area. Fluorescein angiography (fa) of the right eye showed leakage of the optic disc and peripapillary areas, as well as staining and leakage of lymphomatous lesions. A vitreous tap in the right eye was performed and cytology of the vitreous sample revealed large atypical lymphoid cells with scanty basophilic cytoplasm and large segmented nuclei consistent with intraocular large b-cell lymphoma. Lost to follow up and presented 1-year later with new onset decreased vision in the left eye for 2 weeks. The bcva of his right eye was stable at hm while the left eye had declined to 20/63. Slit-lamp examination revealed a quiet anterior chamber bilaterally. Right fundus examination revealed no vitreous cells, an unchanged submacular scar and mottling of the rpe. In the left eye, new 2+ vitreous cell was noted without visible retinal lesions. Oct macula of the left eye showed disruption of ellipsoid zone and an intraretinal vertical hyperreflective lesion located inferotemporally to the fovea. The patient was diagnosed with a presumed second isolated ocular recurrence.
Remission Characteristics
On subsequent follow-up two months after his presentation, visual acuity remained at hm and a significant reduction of intraretinal and subretinal infiltration was noted. Faf of the right eye also revealed a decrease in the hypo-autofluorescence area corresponding to an apparent improvement. Additionally, oct macula of the right eye showed thinning and atrophy. Second admission: on follow-up visits, he reported spontaneous improvement of vision associated with improved visual acuity to 20/40 in the left eye. Serial oct demonstrated reconstitution of the ellipsoid zone. In addition, the vertical hyper- reflective lesion appeared to regress and resolved by 1 month (fig. 3c and d). One month later, bcva in the left eye improved to 20/32 and there were no signs of recurrence at subsequent appointments.
Treatment & Mechanisms
Clinical Treatment
Vitreous tap