OBJECTIVE: To report the clinical, electrophysiologic, and immunopathologic findings in a patient with progressive retinal degeneration associated with Waldenström's macroglobulinemia. DESIGN: Case report with immunohistochemical studies. METHODS: A 46-year-old female with elevated serum immunoglobulin M (IgM) levels complained of photophobia and photopsias. Complete ophthalmic examinations including electrophysiologic testing and hematologic evaluations were performed over a 3-year period. Immunohistochemical studies to determine the presence of serum antiretinal antibodies were evaluated using confocal microscopy. RESULTS: Examination of the patient's fundus remained normal, although there was deterioration in her visual acuity, visual field, and electroretinogram over the follow-up period. Waldenström's macroglobulinemia was diagnosed by bone marrow biopsy. Indirect immunohistochemistry revealed reactivity of the patient's serum against the photoreceptor-connecting cilium. CONCLUSION: The paraneoplastic retinopathy associated with Waldenström's macroglobulinemia in this patient is presumed to result from antibodies of the IgM subtype reacting to proteins in the retinal photoreceptors.
OBJECTIVE: To report the clinical, electrophysiologic, and immunopathologic findings in a patient with progressive retinal degeneration associated with Waldenström's macroglobulinemia. DESIGN: Case report with immunohistochemical studies. METHODS: A 46-year-old female with elevated serum immunoglobulin M (IgM) levels complained of photophobia and photopsias. Complete ophthalmic examinations including electrophysiologic testing and hematologic evaluations were performed over a 3-year period. Immunohistochemical studies to determine the presence of serum antiretinal antibodies were evaluated using confocal microscopy. RESULTS: Examination of the patient's fundus remained normal, although there was deterioration in her visual acuity, visual field, and electroretinogram over the follow-up period. Waldenström's macroglobulinemia was diagnosed by bone marrow biopsy. Indirect immunohistochemistry revealed reactivity of the patient's serum against the photoreceptor-connecting cilium. CONCLUSION: The paraneoplastic retinopathy associated with Waldenström's macroglobulinemia in this patient is presumed to result from antibodies of the IgM subtype reacting to proteins in the retinal photoreceptors.