高级检索

    IgD型多发性骨髓瘤的临床特征、疗效及生存分析

    Clinical characteristics, efficacy, and survival analysis of IgD-type multiple myeloma

    • 摘要: 目的 探讨IgD型多发性骨髓瘤(MM)的临床特征、疗效、生存及预后因素。方法 回顾性分析2016年3月—2024年8月徐州医科大学附属医院56例IgD型MM患者的临床资料,分析其疗效和生存预后。结果 56例患者中,男性40例(71.4%),≤65岁44例(78.6%),λ轻链型53例(94.3%),ISSⅢ期18例(48.7%,18/37),伴有细胞遗传学异常23例(85.2%,23/27),其中1q21+最常见(74.1%,20/27)。37例接受蛋白酶体抑制剂(PIs)为基础方案治疗者,疗效评估为部分缓解(PR)及以上者34例,总反应率(ORR)达91.9%;完全缓解(CR)及以上者20例(54.1%)。中位随访时间41个月,中位无进展生存期(PFS)和中位总生存期(OS)分别为24和67个月。单因素Cox分析显示,年龄>65岁、血小板<100×109/L、肌酐≥177 μmol/L、乳酸脱氢酶>250 U/L、骨髓浆细胞比例≥60%、17p-是OS的不良预后因素,其中17p-也是PFS的不良预后因素及OS的独立预后因素。10例接受自体造血干细胞移植(ASCT)者,中位OS(67个月)略优于未移植组(52个月,P=0.068)。15例接受CAR-T治疗者,中位OS(未达到)优于未接受组(52个月,P=0.036),CAR-T后中位PFS为28个月。结论 IgD型MM以≤65岁男性,λ轻链表达为主,初诊分期较晚,常伴细胞遗传学异常;17p-是OS的独立不良预后因素;新药时代下,ASCT和CAR-T治疗有助于延长患者生存期。

       

      Abstract: Objective To investigate the clinical characteristics, treatment efficacy, survival outcomes, and prognostic factors in patients with IgD multiple myeloma (MM). Methods Retrospective analysis was conducted on the clinical data of 56 IgD-type MM patients who were treated at the Affiliated Hospital of Xuzhou Medical University between March 2016 and August 2024. Their treatment efficacy and survival outcomes were analyzed. Results Among the 56 patients, 40 were male (71.4%) and 44 were aged ≤65 years (78.6%). The λ light chain type was observed in 53 cases (94.3%). Of 37 staged patients, 18 were in ISS stage Ⅲ (48.7%). Cytogenetic abnormalities were present in 23 of 27 tested patients (85.2%), with 1q21+ being the most common (74.1%, 20/27). Among 37 patients treated with proteasome inhibitor (PI)-based regimens, 34 achieved partial remission (PR) or better, with an overall response rate (ORR) of 91.9%. Complete remission (CR) or better was achieved in 20 cases (54.1%). The median follow-up time was 41 months, while the median progression-free survival (PFS) and median overall survival (OS) were 24 and 67 months, respectively. Univariate Cox analysis showed that age >65 years, platelet count <100×109/L, creatinine ≥177 μmol/L, lactate dehydrogenase >250 U/L, bone marrow plasma cell proportion ≥60%, and 17p deletion were adverse prognostic factors for OS, with 17p deletion also being an adverse factor for PFS and an independent prognostic factor for OS. Ten patients underwent autologous stem cell transplantation (ASCT), with a median OS of 67 months, slightly better than the non-transplant group (52 months, P=0.068). Fifteen patients received CAR-T therapy; the median OS was not reached and was superior to that of the non-CAR-T group (52 months, P=0.036). The median PFS after CAR-T was 28 months. Conclusions IgD-type MM predominantly occurs in males ≤65 years old, mainly with λ light chain expression. Patients often present with advanced disease at diagnosis and exhibit frequent cytogenetic abnormalities. The 17p deletion is an independent adverse prognostic factor for OS. In the era of novel therapies, ASCT and CAR-T therapy may contribute to prolonged survival in patients with IgD MM.

       

    /

    返回文章
    返回