Localized conjunctival amyloidosis associated with extranodal lymphoma

A 62-year-old man was found to have a large asymptomatic amyloid nodule of the conjunctiva. Within the next year, a diffuse mixed small and large cell lymphoma of his scapula developed with amyloid in its stroma. No abnormal serum or urine protein was found. Subsequently, rib and paraspinal masses appeared. After irradiation and chemotherapy, the patient died of bronchopneumonia, 3 years after onset. By immunostaining, the amyloid of both conjunctiva and scapular tumor was of polyclonal immunoglobulin (AL) type, most prominently IgG and lambda chains, with lesser staining for IgA and kappa chains. The tumor cells showed a similar pattern. To our knowledge, this is the first report of systemic lymphoma and localized conjunctival amyloidosis without a serum paraprotein, and the first such association with a lymphoma of this cell type. Immunotyping of conjunctival amyloidosis should be performed to guide the workup for possible systemic disease.