15 例 IgD 多发性骨髓瘤患者的特征及实验室在诊断中的关键作用:回顾性研究报告和文献综述

J. Intra, Sara Pezzatti, R. Brivio, Monica Carpenedo, Rita Romano, Nadia Spinoni, Marco Casati
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引用次数: 0

摘要

免疫球蛋白D(IgD)骨髓瘤是多发性骨髓瘤(MM)中一种不常见的亚型,占病例的1%-2%。与其他亚型的多发性骨髓瘤患者相比,IgD型骨髓瘤患者的预后和存活率较低。我们对2008年至2022年期间确诊的15名IgD MM患者(9男6女)进行了回顾性研究,以调查确诊时的临床和生化特征、细胞遗传学改变和存活时间。中位年龄为69岁,骨质病变、肾功能损害、本-琼斯蛋白尿和血清低密度脂蛋白胆固醇(LDH)升高的频率较高。血清钙水平在参考范围内。在对蛋白质电泳图谱进行评估时,有九名患者的血清中检测不到单克隆蛋白。荧光原位细胞遗传学分析表明,最常见的异常是 13q 缺失和 IGH 重排。接受新化疗药物(免疫调节剂、蛋白酶体抑制剂)治疗的患者,无论是否进行自体干细胞移植,中位生存率都较高。实验室在单克隆IgD检测以及IgD MM病例的监测和研究中发挥着重要作用,它增进了人们对该疾病的了解,从而改善了患者的预后。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Characteristics of 15 Subjects Affected by IgD Multiple Myeloma and the Key Role of the Laboratory in Diagnosis: A Retrospective Study Report and Literature Review
Immunoglobulin D (IgD) myeloma represents an uncommon subtype of multiple myeloma (MM), accounting for 1–2% of cases. Subjects affected by IgD MM have been demonstrated to have an inferior outcome and survival compared to those with other MM subtypes. A retrospective study was conducted on 15 patients (9 males and 6 females) diagnosed from 2008 to 2022 with IgD MM, in order to investigate the clinical and biochemical features at the moment of diagnosis, cytogenetic alterations, and survival times. The median age was 69 years, and higher frequencies of bone lesions, renal impairments, Bence–Jones proteinuria, and increased serum LDH were observed. Serum calcium levels were in the reference ranges. In the assessment of protein electrophoresis patterns, nine patients had a serum monoclonal protein that was not detectable. A cytogenetic analysis via fluorescence in situ demonstrated that the most common abnormalities were the deletion of 13q and IGH rearrangements. Patients treated with new chemotherapeutic drugs (immunomodulators, proteasome inhibitors), with or without autologous stem cell transplantation presented a higher median survival. The fundamental role of the laboratory in monoclonal IgD detection and the monitoring and studying of IgD MM cases enhances the knowledge of this disease, thus improving patient outcomes.
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