Macular retinoschisis in a patient with multiple lymphoproliferative malignancies

Abstract

Purpose: To describe a case of macular retinoschisis in a patient with multiple systemic lymphoproliferative disorders and the challenges of accurate diagnosis of uveal lymphoma. Observations: A 66-year-old male presented with unilateral macular retinoschisis and choroidal thickening in the right eye had an unrevealing systemic work-up for inflammatory or neoplastic processes. The patient eventually developed mild vitritis and a diagnostic vitrectomy was performed. Flow cytometry of the vitreous showed clonal expansion consistent with a mature B cell lymphoma of the uveal tract. Repeat systemic work-up including bone marrow biopsy, however, revealed two additional systemic conditions: Waldenstrom macroglobulinemia (WM) and monoclonal B cell lymphocytosis. Based on B cell gene rearrangement testing, the choroidal lymphoma was found to be distinct from the systemic lymphoma. In total, the patient had three lymphoproliferative disorders. Soon after the diagnosis, the left eye also developed choroidal thickening. The patient received orbital external beam radiation and zanubrutinib to treat the systemic WM and the choroidal lymphoma with improvement of macular retinoschisis and choroidal thickening. Conclusions: We present a case of macular retinoschisis in a patient with choroidal lymphoma and multiple systemic lymphoproliferative disorders. Both the systemic disease and the uveal lymphoma contributed to the unique ocular findings in this patient. Our case highlights the challenges of uveal lymphoma diagnosis and the importance of tissue biopsy, systemic work-up, and close monitoring.