Angioimmunoblastic lymphadenopathy. Long-term follow-up study.

The clinical course of 41 previously reported patients with angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) on whom follow-up information has been obtained for five or more years is described. Of the 41 patients, 27 achieved a complete remission (CR). The durations of the CR ranged from two to 214 months, with a median of 48 months. Nine of these 27 complete responders are still alive and well without evidence of disease, whereas the remaining 18 patients have died of pneumonia, septicemia, immunoblastic lymphoma, or unrelated causes. These 27 patients had a significantly longer median survival (51 mos) than did the 14 patients who had partial or no response (9 mos) (P = 0.0006). Only two of these 14 patients who did not initially achieve a CR are alive (survivals, 66 months and 70 months). There was a trend suggesting that patients who received combination chemotherapy which included prednisone had a slightly longer survival than did the remaining patients (P = 0.087). Lymphocytopenia was evident in a higher proportion of dead patients than in those who remained alive (P = 0.089).