Angioimmunoblastic lymphadenopathy with dysproteinemia. Circulating immune complexes and the review of 18 cases

Eighteen patients with angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) have been analyzed by reviewing all available biopsy, laboratory, and clinical data. Because of features suggesting the presence of circulating immune complexes (CIC), CIC serial sample determinations were performed throughout their disease in available patients. Age, sex, and clinical and laboratory characteristics were consistent with previously reported series. Six of 18 (33%) patients having a drug exposure associated with onset or exacerbation of symptoms demonstrated a significantly decreased survival (P less than 0.02). Achievement of complete remission was a significant indicator of longevity (P less than 0.001). Only one patient (6%) developed diffuse histiocytic lymphoma. Elevated CIC were detected in the four patients tested. In two patients fluctuating CIC levels could clearly be correlated to clinical remission or exacerbation. Despite the small number of patients tested, it was concluded tha CIC may provide useful information for therapy selection, prediction of relapse, and further insight into pathogenetic mechanisms in AILD.