Waldenström's macroglobulinemia-associated retinopathy.
OBJECTIVE
To report the clinical, electrophysiologic, and immunopathologic findings in a patient with progressive retinal degeneration associated with Waldenström's macroglobulinemia.


METHODS
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.


CONCLUSIONS
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.
Sen
H Nida
HN
Laboratory of Immunology, National Eye Institute, National Institutes of Health, Building 10, Room 10N112, 9000 Rockville Pike, Bethesda, MD 20892-1857, USA.
Chan
Chi-Chao
CC
Caruso
Rafael C
RC
Fariss
Robert N
RN
Nussenblatt
Robert B
RB
Buggage
Ronald R
RR
D001323:Autoantibodies / Q000097:blood; D001324:Autoantigens / Q000276:immunology; D004596:Electroretinography; D005260:Female; D019084:Fluorescent Antibody Technique, Indirect; D006801:Humans; D007075:Immunoglobulin M / Q000097:blood; D018613:Microscopy, Confocal; D008875:Middle Aged; D010257:Paraneoplastic Syndromes / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D020795:Photophobia / Q000209:etiology; D012160:Retina / Q000276:immunology / Q000503:physiopathology; D012164:Retinal Diseases / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D014792:Visual Acuity / Q000502:physiology; D014794:Visual Fields / Q000502:physiology; D008258:Waldenstrom Macroglobulinemia / Q000150:complications* / Q000175:diagnosis
D001323:Autoantibodies; D001324:Autoantigens; D007075:Immunoglobulin M