UCL Cancer Institute, London, United Kingdom.
UCL Great Ormond Street Institute of Child Health, London, United Kingdom.
Blood Adv. 2022 Sep 13;6(17):5113-5123. doi: 10.1182/bloodadvances.2022007608.
Immunoglobulin D (IgD) myeloma is a subtype often considered to have adverse features and inferior survival, but there is a paucity of data from large clinical studies. We compare the clinical characteristics and outcomes of patients with IgD myeloma from UK phase 3 myeloma trials analyzed in 2 groups: old (1980-2002) and recent (2002-2016) clinical trials, based on the time of adoption of novel myeloma therapies. Patients with IgD myeloma comprised 44 of 2789 (1.6%) and 70 of 5773 (1.2%) of the old and recent trials, respectively. Overall, IgD myeloma was associated with male predominance, low-level paraproteinemia (<10g/L), and λ light chain preference. The frequency of ultra-high-risk cytogenetics was similar in IgD myeloma compared with other subtypes (4.3% vs 5.3%, P > .99). Despite the old trial series being a younger group (median age: 59 vs 63 years, P = .015), there was a higher frequency of bone lesions, advanced stage at diagnosis, worse performance status, and severe renal impairment compared with the recent trials. Furthermore, the early mortality rate was significantly higher for the old trial series (20% vs 4%, P = .01). The overall response rate following induction therapy was significantly higher in the recent trials (89% vs 43%, P < .0001), and this was consistent with improved median overall survival (48 months; 95% confidence interval [CI] 35-67 months vs 22 months; 95% CI, 16-29 months). Survival outcomes for IgD myeloma have significantly improved and are now comparable to other myeloma types because of earlier diagnosis, novel therapies, and improved supportive care. This trial was registered at clinicaltrials.gov as # NCT01554852.
免疫球蛋白 D（IgD）骨髓瘤通常被认为具有不良特征和较差的生存预后，但来自大型临床研究的数据很少。我们比较了英国 3 期骨髓瘤试验中分析的 2 组 IgD 骨髓瘤患者的临床特征和结局：旧（1980-2002 年）和新（2002-2016 年）临床试验，根据新型骨髓瘤疗法的采用时间进行分组。旧和新临床试验中分别有 44 例（1.6%）和 70 例（1.2%）患者患有 IgD 骨髓瘤。总体而言，IgD 骨髓瘤与男性为主、低水平的副蛋白血症（<10g/L）和 λ 轻链偏好有关。与其他亚型相比，IgD 骨髓瘤中超高风险细胞遗传学的发生率相似（4.3%比 5.3%，P>.99）。尽管旧试验系列的年龄较小（中位年龄：59 岁比 63 岁，P=.015），但与新试验相比，骨病变、诊断时的晚期、较差的体能状态和严重的肾功能损害的发生率更高。此外，旧试验系列的早期死亡率显著更高（20%比 4%，P=.01）。新试验中诱导治疗后的总缓解率显著更高（89%比 43%，P<.0001），这与中位总生存期的改善一致（48 个月；95%置信区间 [CI] 35-67 个月比 22 个月；95% CI，16-29 个月）。由于更早的诊断、新型疗法和更好的支持性护理，IgD 骨髓瘤的生存结局得到了显著改善，现在与其他骨髓瘤类型相当。该试验在 clinicaltrials.gov 上注册为 #NCT01554852。
