Characteristic OCT Pattern in Waldenstrom Macroglobulinemia.

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

Hypocomplementaemic urticarial vasculitis syndrome: a mimicker of systemic lupus erythematosus.

A middle aged female patient presented with generalised palpable purpura associated with intense pruritus along with subconjunctival haemorrhage and orbital inflammation. There was extensive dermographism. Other systemic examinations were within normal limits. Haematological profile was normal except raised D-dimer. Skin biopsy revealed the presence of leucocytoclastic vasculitis. Antinuclear antibody was positive in a titre of 1 : 160, but antidouble-stranded DNA was negative. Urine examination revealed haematuria and proteinuria. Complement C3, C4 and C1q levels were decreased with the presence of anti-C1q antibody. There was a diagnostic dilemma between systemic lupus erythematosus and hypocomplementaemic urticarial vasculitis syndrome. However, as the patient did not fulfil the American College of Rheumatology criteria for systemic lupus erythematosus, but fulfilled all the criteria for hypocomplementaemic urticarial vasculitis syndrome, the case was finally diagnosed as hypocomplementaemic urticarial vasculitis syndrome and treated accordingly with favourable outcome.