Purpose: To analyze the clinical and laboratory characteristics, response to therapy, and survival in 53 patients with immunoglobulin D (IgD) multiple myeloma (MM) from a single institution. Patients and Methods: Records of all Mayo Clinic patients with IgD seen between January 1, 1965 and December 31, 1992 were reviewed. Survival curves were plotted according to the Kaplan-Meier method and statistically compared using the log-rank test. Results: The main presenting features were bone pain (72%), fatigue (36%), weight loss (32%), extramedullary plasmacytomas (19%), and associated amyloidosis (19%). Renal function impairment and hypercalcemia were present in 33% and 22% of patients, respectively. The serum electrophoretic pattern showed and M-spike in only 60% of the patients, the remaining having either hypogammaglobulinemia or a normal-appearing pattern. Bence Jones proteinuria was identified in 96%. The type of light chain was lambda in 60% of the patients, kappa in 38%, and indeterminate in 2%. Among 45 patients assessable for response, the objective response rate was 58%. The median duration of survival in the whole series was 21 months. There was a trend for loner survival in patients given combination chemotherapy (median, 20 v 64 months; P = .09). The proportions of patients alive at 3 and 5 years were 36% and 21% respectively. Conclusion: Patients with IgD myeloma usually present with a small or no visible monoclonal spike on serum electrophoresis and light-chain proteinuria, thus resembling Bence Jones myeloma. Response to therapy is similar to that observed in other myeloma types. Although the median duration of survival is shorter than that currently achieved in patients with myeloma, one third and one fifth survive for more than 3 and 5 years, respectively.
