Characteristic OCT Pattern in Waldenstrom Macroglobulinemia

To report characteristic optical coherence tomography (OCT) patterns as a guide in diagnosis of Waldenstrom macroglobulinemia in a patient presenting with bilateral vision loss.

A 60-year-old male patient presented with bilateral sudden-onset vision loss and low-grade fever. The patient had bilateral exudative retinal detachment involving the macula. Spectral domain OCT revealed neurosensory detachment along with a very regular and harmonious cystoid change in the outer retina as well as deposits around photoreceptors. The patient had lymphadenopathy and hepatosplenomegaly. Serum protein electrophoresis showed the presence of M band in the gamma globulin region, which, on immune fixation, showed a monoclonal band at the "IgM and K" region. Infiltration by lymphoplasmacytic proliferative cells with secondary myelofibrosis was seen in the bone marrow biopsy specimen. The immunophenotype scattergram of lymphoplasmacytic proliferative cells of bone marrow showed no light-chain expression, and the cells were brightly positive for CD19, CD5, and CD20. Quantitative assay of immunoglobulin M was 10,057 mg/dL.

Spectral domain OCT showing a harmonious and regular cystoid pattern and deposits around photoreceptors was a guide in our diagnosis of this case of Waldenstrom macroglobulinemia presenting with retinal detachment and retinal deposits.