间变性多发性骨髓瘤:病例系列和文献回顾

Jian Wu, E.Y.-L. Chu, Cristiana Costa Chase, T. Choi, C. Gasparetto, K. Young, Yubin Kang
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引用次数: 1

摘要

背景:间变性多发性骨髓瘤(AMM)是一种非常罕见但独特的多发性骨瘤(MM)亚型,预后极差。由于其稀有性,AMM缺乏详细的描述和明确的定义。此外,在治疗方面还没有达成共识,有证据表明AMM对几种新疗法的反应不佳。我们进行了文献综述和回顾性病例系列,以确定AMM的临床特征、病理特征和结果。病例介绍:对1983年以来发表的AMM病例报告和病例系列进行了系统的提取和回顾。自1983年以来,PUBMED共报告了52名AMM患者,包括26名男性(50%)和26名女性(50%)。年龄29~85岁,平均57.02岁。大多数患者表现为骨痛(23,44.2%)、疲劳(18,34.6%)、浆细胞瘤(18,3.46%)和体重减轻(7,13.5%)。患者的中位生存期为4个月。为了研究AMM患者在当前治疗时代的结果,我们对2012年12月至2021年7月在我们研究所诊断的14名AMM患者进行了回顾性分析。我们的回顾性病例系列包括12名男性(85.7%)和2名女性(14.3%),平均年龄59岁。我们的大多数AMM患者表现出溶骨病变是一种常见的表现。常见的细胞遗传学异常为1q扩增。所有患者均接受了由蛋白酶体抑制剂和/或免疫调节剂组成的标准联合化疗,其中一半患者接受了自体造血干细胞移植。我们的14名AMM患者的中位无进展生存期(PFS)和总生存期(OS)分别为0.84年和1.52年,这明显低于2003-2013年在我们研究所接受治疗的常规MM患者,后者的PFS为2.28年,OS为4.92年。结论:AMM是MM的一种非常罕见的、形态上独特的变体。它具有不良的细胞遗传学和侵袭性过程。它通常对标准化疗具有耐药性,并且存活率极低。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Anaplastic Multiple Myeloma: Case Series and Literature Review
Background: Anaplastic multiple myeloma (AMM) is a very rare but distinct subtype of multiple myeloma (MM) with an extremely poor prognosis. Due to its rarity, AMM lacks detailed descriptions and clear definitions. Moreover, there is no consensus on the treatment and evidence suggests that AMM responds poorly to several novel therapies. We conducted a literature review and retrospective case series to determine clinical characteristics, pathological features, and outcomes of AMM. Case Presentation: Published case reports and case series of AMM since 1983 were systematically extracted and reviewed. A total of 52 patients with AMM were reported in the PUBMED since 1983, including 26 males (50%) and 26 females (50%). The age ranged from 29 years old to 85 years old, with a mean age of 57.02 years old. Most of the patients presented with bone pain (23, 44.2%), fatigue (18, 34.6%), plasmacytoma (18, 34.6%) and weight loss (7, 13.5%). The median survival of the patients was 4 months. To investigate the outcomes of patients with AMM in the current era of treatment, a series of 14 patients with AMM diagnosed at our institute between December 2012 and July 2021was retrospectively analyzed. Our retrospective case series consisted of 12 males (85.7%) and 2 females (14.3%), with a mean age of 59 years old. Most of our AMM patients displayed bone lytic lesions as a common manifestation. The common cytogenetic abnormality was 1q amplification. All patients received standard combination chemotherapy consisting of proteasome inhibitors and/or immunomodulatory agents, and half of the patients underwent autologous hematopoietic stem cell transplantation. The median progression-free survival (PFS) and overall survival (OS) for our 14 AMM patients were 0.84 years and 1.52 years, respectively, which was significantly worse than the regular MM patients treated at our institute from 2003–2013 who had a PFS of 2.28 years and OS of 4.92 years. Conclusions: AMM is a very rare, morphologically distinct variant of MM. It has adverse cytogenetics and an aggressive course. It is often resistant to standard chemotherapy and presents with an extremely low survival rate.
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