Bladé J, Lust J A, Kyle R A
Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, MN 55905.
J Clin Oncol. 1994 Nov;12(11):2398-404. doi: 10.1200/JCO.1994.12.11.2398.
To analyze the clinical and laboratory characteristics, response to therapy, and survival in 53 patients with immunoglobulin D (IgD) multiple myeloma (MM) from a single institution.
Records of all Mayo Clinic patients with IgD MM seen between January 1, 1965 and December 31, 1992 were reviewed. Survival curves were plotted according to the Kaplan-Meier method and statistically compared using the log-rank test.
The main presenting features were bone pain (72%), fatigue (36%), weight loss (32%), extramedullary plasmacytomas (19%), and associated amyloidosis (19%). Renal function impairment and hypercalcemia were present in 33% and 22% of patients, respectively. The serum electrophoretic pattern showed an M-spike in only 60% of the patients, the remaining having either hypogammaglobulinemia or a normal-appearing pattern. Bence Jones proteinuria was identified in 96%. The type of light chain was lambda in 60% of the patients, kappa in 38%, and indeterminate in 2%. Among 45 patients assessable for response, the objective response rate was 58%. The median duration of survival in the whole series was 21 months. There was a trend for longer survival in patients given combination chemotherapy (median, 20 v 64 months; P = .09). The proportions of patients alive at 3 and 5 years were 36% and 21%, respectively.
Patients with IgD myeloma usually present with a small or no visible monoclonal spike on serum electrophoresis and light-chain proteinuria, thus resembling Bence Jones myeloma. Response to therapy is similar to that observed in other myeloma types. Although the median duration of survival is shorter than that currently achieved in patients with myeloma, one third and one fifth survive for more than 3 and 5 years, respectively.
分析来自单一机构的53例免疫球蛋白D（IgD）多发性骨髓瘤（MM）患者的临床和实验室特征、治疗反应及生存情况。
回顾了1965年1月1日至1992年12月31日期间在梅奥诊所就诊的所有IgD MM患者的记录。根据Kaplan-Meier方法绘制生存曲线，并使用对数秩检验进行统计学比较。
主要临床表现为骨痛（72%）、疲劳（36%）、体重减轻（32%）、髓外浆细胞瘤（19%）和相关淀粉样变性（19%）。分别有33%和22%的患者出现肾功能损害和高钙血症。血清电泳图谱仅在60%的患者中显示M峰，其余患者表现为低丙种球蛋白血症或正常图谱。96%的患者检测到本周氏蛋白尿。60%的患者轻链类型为λ，38%为κ，2%不确定。在45例可评估反应的患者中，客观反应率为58%。整个系列的中位生存时间为21个月。接受联合化疗的患者生存时间有延长趋势（中位时间分别为20个月和64个月；P = 0.09）。3年和5年存活患者的比例分别为36%和21%。
IgD骨髓瘤患者血清电泳通常显示单克隆峰小或无，且有轻链蛋白尿，因此类似于本周氏骨髓瘤。治疗反应与其他骨髓瘤类型相似。虽然中位生存时间短于目前骨髓瘤患者的生存时间，但分别有三分之一和五分之一的患者存活超过3年和5年。
