Institute of Hematology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, 1277 Jiefang Dadao, Wuhan, 430022, China.
Ann Hematol. 2019 Apr;98(4):963-970. doi: 10.1007/s00277-018-3582-4. Epub 2019 Jan 4.
Immunoglobulin D (IgD) myeloma is a rare subtype that used to lead to a poor outcome. To investigate the current clinical features, cytogenetic changes and survival of patients with IgD myeloma under novel treatments, we analysed 47 patients with IgD myeloma, 31 men and 16 women, with a median age of 54.5 years. We found that IgD myeloma was associated with higher frequencies of anaemia, renal failure, and hypercalcemia and higher levels of serum LDH compared with non-IgD myeloma. More than 90% of patients with IgD myeloma had at least one cytogenetic abnormality demonstrated by fluorescence in situ hybridisation (FISH). IGH translocations were the most common abnormalities, which were mainly caused by t(11;14). Moreover, 36.2% of patients were at the Revised International Staging System (RISS) stage III when diagnosed. Those patients had significantly shorter PFS and OS compared with patients at RISS stages I and II. In conclusion, IgD myeloma has specific clinical characteristics. The RISS grade was shown to be a simple and effective method to predict the prognosis of patients with IgD myeloma.
免疫球蛋白 D（IgD）骨髓瘤是一种罕见的亚型，过去预后较差。为了研究新型治疗方案下 IgD 骨髓瘤患者的当前临床特征、细胞遗传学改变和生存情况，我们分析了 47 例 IgD 骨髓瘤患者，其中男 31 例，女 16 例，中位年龄 54.5 岁。我们发现与非 IgD 骨髓瘤相比，IgD 骨髓瘤患者贫血、肾衰竭和高钙血症的发生率更高，血清 LDH 水平更高。超过 90%的 IgD 骨髓瘤患者通过荧光原位杂交（FISH）检测至少存在一种细胞遗传学异常。IGH 易位是最常见的异常，主要由 t(11;14)引起。此外，36.2%的患者在确诊时处于修订后的国际分期系统（RISS）III 期。与 RISS 分期 I 期和 II 期的患者相比，这些患者的 PFS 和 OS 明显更短。总之，IgD 骨髓瘤具有特定的临床特征。RISS 分级是预测 IgD 骨髓瘤患者预后的一种简单有效的方法。
