MULTIPLE AUTOIMMUNE MANIFESTATIONS IN MONOCLONAL GAMMOPATHY OF UNDETERMINED SIGNIFICANCE AND CHRONIC LYMPHOCYTIC-LEUKEMIA

In 18 cases of monoclonal gammopathy of undetermined significance, MGU S (monoclonal gammopathy of undetermined significance), admitted for d iagnosed or suspected peripheral neuropathy, II patients showed other co-existing autoimmune manifestations. Two had POEMS syndrome (polyneu ropathy, organomegaly, endocrinopathy, M-component, and skin symptoms) , the others mainly endocrinopathy and polyclonal pseudolymphoma. Ther e were 13 cases of sensorimotor neuropathy, two cases of neuritis, whi le neuropathy could not be confirmed in three cases. Compared with a r etrospective review of autoimmunity in a randomly selected CLL (chroni c lymphocytic leukemia) cohort of 115 patients, 13 out of 42 patients with clinical and/or laboratory features of autoimmunity showed co-exp ression of autoimmune signs, the dominating traits being Coombs' posit ive AIHA (auto immune hemolytic anemia), platelet autoantibodies, endo crinopathy mainly associated with the thyroid gland, serological and/o r rheumatological symptoms, but only one case of sensorimotor neuropat hy. Viewed from a current model of acquired autoimmunity it is perhaps not surprising that such autoimmunity is seen predominantly in patien ts with monoclonal gammopathy. Thus, a high concentration of cross-rea cting polyreactive autoantibodies related to the M-component might be present in these patients. Furthermore, quantitative defects of the im munoglobulins including the hypogammaglobulinemia associated with M-co mponents can presumably give rise to a defect of the antiidiotypic net work's regulation of natural autoantibodies and autoimmune manifestati ons in vivo. Such autoimmune manifestations, which are easily overlook ed in CLL may call for additional treatment with immunosuppression and /or intravenous, polyclonal IgG.