A 44 year old woman presented with discoid lupus erythematosus and then chronic lymphocytic leukemia of the B cell type with macroglobulinemia developed. Immunologic studies revealed suppressed thymus-dependent cell function, increased bone marrow-dependent cells of the immunoglobulin M (IgM) class and increased null cells.The pathogenesis and relation of lupus erythematosus to B cell malignancy with hyperimmunoglobulinemia are discussed.