IMMUNOGLOBULIN-D MULTIPLE-MYELOMA - PRESENTING FEATURES, RESPONSE TO THERAPY, AND SURVIVAL IN A SERIES OF 53 CASES
IMMUNOGLOBULIN-D MULTIPLE-MYELOMA - PRESENTING FEATURES, RESPONSE TO THERAPY, AND SURVIVAL IN A SERIES OF 53 CASES
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DOI：
10.1200/jco.1994.12.11.2398
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发表时间：
1994-11-01
影响因子：
45.3
通讯作者：
KYLE, RA
中科院分区：
文献类型：
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作者：
BLADE, J;LUST, JA;KYLE, RA
目的：分析53例免疫球蛋白D（IgD）多发性骨髓瘤（MM）从一个单一institution.Patients和方法：所有马约诊所的IgD患者的记录，1965年1月1日和1992年12月31日之间看到的临床和实验室特征，治疗反应和生存进行了审查。生存曲线绘制根据Kaplan-Meier方法和统计学比较使用log-rank test.Results：主要表现为骨痛（72%），疲劳（36%），体重减轻（32%），髓外浆细胞瘤（19%），和相关的淀粉样变性（19%）。分别有33%和22%的患者出现肾功能损害和高钙血症。血清电泳模式显示，M-穗只有60%的患者，其余的要么低丙种球蛋白血症或一个正常的外观模式。Bence Jones蛋白尿检出率为96%。60%的患者轻链类型为λ，38%为κ，2%为不确定。在45例可评估缓解的患者中，客观缓解率为58%。整个系列的中位生存期为21个月。接受联合化疗的患者有生存期延长的趋势（中位数，20 vs 64个月; P = 0.09）。3年和5年存活的患者比例分别为36%和21%.Conclusion：IgD骨髓瘤患者通常存在一个小的或没有可见的单克隆尖峰血清电泳和轻链蛋白尿，从而类似于Bence Jones骨髓瘤。对治疗的反应与在其他骨髓瘤类型中观察到的相似。尽管中位生存期比目前骨髓瘤患者的中位生存期短，但三分之一和五分之一的患者分别存活3年和5年以上。
Purpose: 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.Patients and Methods: Records of all Mayo Clinic patients with IgD 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.Results: 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 and 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 loner 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.Conclusion: 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.