Objective To explore the clinical characteristics, treatment, survival, and prognostic factors of patients with IgD multiple myeloma (MM).
Methods Clinical data of 25 patients with IgD MM admitted into the Zhongshan Hospital, Fudan University from June 2013 to September 2021 were analyzed. Baseline characteristics, laboratory tests, and cytogenetic features of patients were collected. Clinical efficacy was evaluated according to International Myeloma Working Group (IWMG) criteria. Survival analysis was performed by Kaplan-Meier curves; univariate analysis was performed using Log-rank test, and variables with P < 0.15 were included in multivariate Cox regression analysis.
Results Among the 25 patients with IgD MM, 21 cases (68%) were male, with 21 cases (84%) younger than 65 years old. 24 patients (96%) of IgD MM were of λ light chain type. 21 patients (84%) were in DS-Ⅲ stage and 12 patients (48%) were in ISS-Ⅲ stage. FISH results showed that 77.3% (17/22) of patients had cytogenetic abnormalities. Among them, 14 cases (63.6%) complicated with 1q21 amplification and 9 cases (40.9%) with IgH rearrangement, being the most common abnormalities. All patients were treated with chemotherapy regimens containing proteasome inhibitors. The total overall response rate (ORR) was 95% in 20 efficacy-evaluable patients with complete remission (CR) and stringent CR (sCR) rate accounting for 50%, with 66.7% (6/9) and 36.4% (4/11) achieving CR and sCR in patients with and without ASCT, respectively. The median overall survival (OS) and progression-free survival (PFS) were 46.7 months and 25.3 months in all patients. The median PFS in the transplanted and non-transplanted arms were 46.7 and 21.6 months, respectively (P=0.018). Bortezomib and lenalidomide-contained regimen (VRD) resulted in longer OS and PFS than single novel agent regimen of bortezomib (VCD). Elevated lactate dehydrogenase and creatinine levels were adverse prognostic factors associated with OS and PFS.
Conclusions IgD MM is dominated by λ light chain and more commonly happened in men, younger than 65 years old, and often identified at diagnosis with high-risk cytogenetic abnormalities. The availability of novel agents such as proteasome inhibitors (PIs) can improve the efficacy and prognosis of IgD MM, and autologous stem cell transplantation can still play a role in the era of new agents.
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