Pb2177 Immunoglobulin D Multiple Myeloma in the Era of Bortezomib‐based Regimens: A Single Center Experience

Abstract

chains was shown in 92%, median bone marrow plasma cells was 10.9% (5 patients had associated MM), 12% had unfavorable genetic. Mayo stage was distributed as follows: I (8%), II (20%), III (28%), IV (44%). 20 pts (80%) had cardiac involved and 12 (60%) had typical pattern of delayed gadolinium enhancement in magnetic resonance. Median left ventricle thickness was 16.7 mm. Results: The histological diagnosis was in 28% by renal biopsy, 20% minor salivary gland, 16% fat aspiration, 8% cardiac, and proceeds with mass spectroscopy in 2 patients. Fat aspiration was performed in 15 pts (60%), but only 4 (26%) were positive. 44% received one regimen of treatment [0-6 lines]. Bortezomib and lenalidomide were the most used drugs in first and second line (80% and 32% respectively). 20% underwent ASCT (only 2 directly). 16% received doxycycline.The hematologic and organic response for each line and PFS is shown in table 1. Median PFS was decreasing with Mayo stage (1-2 stage: 16 month, 3: 9.7 months, 4: 7 months. Median OS was 4 years [95% CI 1.5-6.4] with median follow up of 7.8 years, and median OS in patients achieving RC/ VGPR was 4.9 years [95% CI 1.7-8].Summary/Conclusion: 1) Our series PFS and OS are worse comparing with literature, in real world AL amyloidosis is still diagnosed late, in advance Mayo stage (72%), and cardiac involvement. 2) The fat aspiration is not the diagnosis tool in the most cases. 3) Early diagnosis is the key to managing the disease and improves de survival of these patients. 4) It could be recommendable to start therapy, if clinical-analytic suspicious is high, although without histological confirmation, but it has to be provided in further studies.