Chronic lymphocytic leukemia and malignant lymphoma developing in patients with auto-immunity or other III-defined immune stigmata: A review of the literature and report of an additional 9 cases

In this report the association of autoimmunity and immune disorders with lymphoproliferative disorders is extensively reviewed and illustrated by nine new cases of lymphoma and B type chronic lymphocytic leukemia (CLL) preceded by or coexistent with (auto) immune stigmata. In most of the patients these 'paraneoplastic' immune stigmata preceded the diagnosis of lymphoma and CLL by months or years. These cases included two patients with well defined disorders such as cold agglutinin disease and systemic lupus erythematosus (SLE) and seven others with ill defined immunological stigmata ranging from cold urticaria, vasculitis, and myalgia associated with arthralgia, to syndromes approximating SLE and rheumatoid arthritis (RA). Five of these patients developed Non-Hodgkin's lymphoma, 2 CLL and 2 Hodgkin's disease. Some of these autoimmune stigmata disappeared when the patients achieved clinical remission. In the light of the above data it is suggested that patients with these phenomena, be followed carefully for the development of lymphoma or CLL. Current views on the association of autoimmunity and immune disorders with lymphoproliferative disorders are discussed in detail and current statistics for this association are reviewed.