After ASCT single or tandem, five patients obtained CR, three VGPR, two PR and one SD. One patient in PR after HDT/ASCT received maintenance with bortezomib and another patient also in PR received Thalidomide as maintenance treatment both patients maintained PR. The ORR in patients treated with GSK126 purchase HDT/ASCT was 90% after a median follow up of 50 months (range 17-148); median OS, PFS [Figures 1, 2] and DOR are not reached. The log-rank test for DOR was P = 0.23. Figure 1 Overall Survival of HDT/ASCT and CT groups. Figure 2 Progression free survival of HDT/ASCT and CT groups. A progression or relapse was observed in 4/11 (36.4%)
patients treated with HDT/ASCT and in 4/6 (66.7%) of those undergone CT. The log-rank test for PFS was P = 0.10, the hazard ratio was 0.31 (95% CI 0.07-1.40). One patient who received single ASCT was treated with allogeneic transplantation at relapse. Peripheral neurophaty of grade 1-2 was observed in all patients treated with thalidomide and or bortezomib either in induction or in maintenance therapy. All patients with bone disease received bisphosphonates; patients treated with thalidomide, buy CB-839 received aspirin or low molecular-weight heparin as thromboprophylaxis and nobody developed venous thromboembolism. Seven of 17 patients
had died by the time of analysis: four in the group treated with CT and three in the group of HDT/ASCT, 85% of death for disease progression; there were no peritransplant deaths. Comparing OS with log-rank test we obtained P = 0.18,
the hazard ratio was 0.37 (95% CI 0.08-1.68). FISH analysis was available only for 6/17 of cases, in these six patients cytogenetic profile had not statistical significance for OS, PFS or DOR Discussion The clinical features of our patients reported in this study underline the worse characteristics of IgD MM. As in other series described in the literature , we also found an advanced stage and a younger age at presentation, with more aggressive clinical course. In addition, the poor survival of the patients may be selleck chemicals llc associated with problems related to delayed diagnosis [13, 19]. Patients with renal failure of unknown cause, bone pain, small serum M-protein bands, or unidentified Ig isotype should be suspected for IgD MM. However, TCL the underlying tumor biology responsible for the differences between IgD MM and other MM isotypes remains to be defined. IgD MM should be considered a rare subgroup of MM with aggressive features rather than a single parameter of poor prognosis. Jancelewicz et al.  reported that λ light-chains are found in 90% and almost the totality of patients had Bence-Jones proteinuria. A mean survival of 13.7 months from diagnosis, that was worse than the common myelomas, was observed in this study. Bladé et al  reviews outcomes in 53 patients from 1965 to 1992 and observed λ light-chain disease in 60%, Bence Jones proteinuria in 96%, renal failure in 33% and hypercalcemia in 22%.