Cancer-Associated Retinopathy in Non-Hodgkin Lymphoma: The Challenge of Treatment

Introduction Cancer-associated retinopathy (CAR) syndrome is rare paraneoplastic visual syndrome, usually associated with small-cell lung carcinoma. We report, to our knowledge, the first case in the English-language literature of CAR syndrome occurring in a patient with non-Hodgkin lymphoma (NHL) treated with intravenous immunoglobulin (IVIG). Case Report A 62-year-old male was diagnosed with stage IV B-cell follicular NHL treated with 6 cycles of R-CHOP (rituximab plus cyclophosphamide/doxorubicin/vincristine/prednisone). Five weeks after the last cycle, he presented with progressive decrease of vision in both eyes, flickering lights, and night blindness. On examination, corrected visual acuity was 20/80 and 20/100 in the right and left eye, respectively, and color vision was diminished bilaterally. Fundus examination revealed pallor of the optic disks while visual field examination showed profound field constriction. A multifocal electroretinogram revealed dysfunction of both rods and cones characteristic of diffuse photoreceptor degeneration that is supporting the diagnosis of CAR syndrome. Magnetic resonance imaging of the head and orbits showed no signs of metastases or other abnormalities. Western blot analysis of the patient's serum showed a positive antibody reaction with 23-kD component coinciding with the molecular mass of human recoverin. The patient was started on oral prednisone 60 mg daily. Three weeks later he still complained of decreased vision in both eyes. IVIG (400 mg/kg/day) was given for 4 doses. Following the second dose, the patient showed signs of improvement. Four weeks after finishing the full treatment, visual acuity improved to 20/40 in both the right and left eye, with no relapse during the 8-month follow-up period. Discussion To our knowledge, there has been only 1 previous report (in the French-language literature) of CAR syndrome in lymphoma patients. Matus et al described 2 NHL patients with symptoms of CAR, which was confirmed by the presence of anti-recoverin antibodies. Both cases were treated by corticosteroids with only minimal response. Since CAR syndrome is a rare condition, no specific treatment has been shown to be effective. Treating the underlining malignancy has not been shown to result in better visual outcomes, and immunosuppression with oral or intravenous corticosteroid exhibits only a transient or undetectable response. Furthermore, plasmapheresis has not been effective. Conclusion CAR may occur in NHL. IVIG may be an effective option for treating these patients, especially if started early enough before permanent retinal damage occurs.