Immunologic Aberrations in the Di Guglielmo Syndrome

Immunologic studies were performed in 49 patients with the Di Guglielmo syndrome. Although altered immune reactivity has not been previously thought to be a feature of myeloproliferative disorders, more than one-third of the cases showed immunologic aberrations. The abnormalities encountered included overproduction of antibody protein (hypergammaglobulinemia) and an increased tendency to form rheumatoid factor, LE factor (including one case with overt systemic lupus), positive serologic tests for syphilis, and erythrocyte autoantibodies and isoantibodies.

Possible pathogenetic mechanisms are considered. The underlying neoplastic process might directly involve the immunocytes, resulting in exaggerated and nonspecific responses, or in defective self-recognition and thus in the production of autoantibodies. Alternatively, preexisting but "hidden" antigens might be exposed by the proliferative disorder, thus stimulating an antibody response. Finally, and perhaps most likely, antigenic alteration of bone marrow tissue might accompany its neoplastic transformation. Such tissue could be recognized as "not-self" or "foreign" by a qualitatively normal immune system. This would result in the production of abnormal proteins, some of which would be immunologically effective.