Immunologic aberrations in the Di Guglielmo syndrome.

IT IS WELL KNOWN that immunologic disturbances, particularly autoimmunity, are frequently associated with the chronic lymphoproliferative diseases. Indeed, it is sometimes difficult in such cases to be certain where the imm’nunologic disorder ends and the neoplastic process begins.’ Included in the long list of autoimmune phenomena found in this disease group have been autoimmune hemolytic a,iemia,2 autoimmune thrombocytopenic purpura ( ITP ) ,:i rheumatoid arthritis,4 thyroiditis,5 systemic lupus erythematosus, and the Sjogren sv,idronic. \Iahgnant disease of nonhemic origin has also been occasionally marked liv the Ires i ce of altered immunologic reactivity-for example. autoimmuiie hemolytic tnemi t associated xvith ovarian tumors,8 the high incidence of derniatomyositis in patients with carcinoma of the breast, stoinach,it and lung,1 and the occurrence of anerg in some patients with (I ISSCITI ii’iated ca’na’ On the other haildi, a close relationship between disturbed immunologic mechanisms a id the m eloproliferative disorders has not been apparent. In(ICedl, we have often stressed the great frequency of immunologic aberrations, including autoii’nmunitv, in lymphoproliferative disorders, as opposed to the lo ’ incidence of these findings in the myeloproliferative dliseases.’2 In the last few years, however, \ ‘(‘ have been struck by the rather frequent occurrence of autoimmune phenom’nena and abnormally high concentrations of immunoglobulins in a series of p ttieiits with one particttl ir type of myeloproliferative