A CASE OF NON-HODGKINS-LYMPHOMA FOLLOWING LONG-TERM CORTICOSTEROID-THERAPY FOR ADDISONS-DISEASE

A 76-year-old Japanese woman had suffered from fatigue, weight loss, a nd cutaneous hyperpigmentation at the age of 38 years and was diagnose d as having tuberculous Addison's disease. Since then, corticosteroids had been administered effectively as hormonal replacement. At the age of 75 years, the patient presented with a progressive, painless swell ing in the left eyelid due to an ill-defined tumor of rubbery consiste ncy in the superotemporal aspect of the orbit. Computed tomography, ma gnetic resonance imaging, and scintigraphy revealed a wide distributio n of tumors, but not in the adrenal gland, which led to the suspicion of systemic malignant lymphoma. Histopathologic examination of the exc ised orbital tumor was compatible with non-Hodgkin's lymphoma of the B -cell type. We believe this is the first report of Addison's disease p resenting with non-Hodgkin's lymphoma. This disease process was charac terized by the development of a lymphoid malignancy after long-term co rticosteroid therapy to control the adrenal insufficiency, and by the widespread involvement of the lymph nodes and orbit but not the adrena l gland. Corticosteroid-induced abnormal immune state was considered t o be the pathogenesis of this unusual complication. Jpn J Ophthalmol 1 998;42:393-397 (C) 1998 Japanese Ophthalmological Society.