Lupus erythematosus in a patient with amyloidosis, adrenal insufficiency, and subsequent immunoblastic sarcoma. Demonstration of the le phenomenon in the lung

Abstract

A patient with systemic lupus erythematosus of long duration developed secondary amyloidosis and finally died after the additional complication of malignant lymphoproliferative disease. Multiple system involvement, typical serologic findings, and postmortem evidence substantiated the diagnosis of lupus erythematosus. Amyloid deposition was found in several organs, but was notably extensive in the adrenal cortex. This extensive deposition resulted in adrenal insufficiency, which was diagnosed clinically and treated until the patient's death from lymphoma. The particular nature of the malignant lymphoma is emphasized; a distinctive feature was the disappearance of positive lupus erythematosus cells from the buffy coat and the reduction in titers of relevant serologic tests toward the end of the illness. In spite of this reduction, many hematoxylin bodies and abundant complete lupus erythematosus cells were found in the lungs on postmortem examination.