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文章:

多发性骨髓瘤胸膜-肺髓外浆细胞瘤:一家三级医疗中心15年经验总结

Pleuro-Pulmonary Extramedullary Plasmacytomas in Multiple Myeloma: A 15-Year Experience from a Tertiary Center

原文发布日期:20 December 2025

DOI: 10.3390/cancers18010019

类型: Article

开放获取: 是

 

英文摘要:

Background/Objectives: Extramedullary involvement in multiple myeloma represents an aggressive disease phenotype, associated with reduced survival and an unfavorable prognosis. Thoracic manifestations are rare and remain poorly characterized in the literature.Methods: We conducted a retrospective, single-center study at the Fundeni Clinical Institute, including patients diagnosed with multiple myeloma between February 2010 and February 2025. The study cohort consisted of 34 patients with infiltration of the pulmonary parenchyma, pleura, or the presence of myelomatous pleural effusion. Diagnosis was confirmed using a combination of imaging modalities (computed tomography or magnetic resonance imaging), cytological examination, immunophenotyping, and histopathological confirmation whenever feasible.Results: Out of a total of 2012 patients with multiple myeloma, the incidence of pleuro-pulmonary extramedullary involvement was 1.6%. The median age at diagnosis was 58 years. Pleuro-pulmonary disease was present at initial diagnosis in 26.5% of cases, while 73.5% developed it at relapse. The most common presentation involved combined pleural involvement and myelomatous effusion (70.6%). Adverse prognostic markers included elevated β2-microglobulin levels (in over 80% of cases) and increased lactate dehydrogenase (LDH) in approximately 50%. Cytogenetic abnormalities such as del(17p), t(4;14), t(14;16), t(11;14), and 1q gain were identified. The median overall survival (OS) from the diagnosis of pleuro-pulmonary extramedullary disease was 16 months, with a 2-year survival rate of 25%. No patient survived beyond 5 years. The median progression-free survival (PFS) was 9 months.Conclusions: Our findings confirm the aggressive clinical course and poor prognosis of these disease manifestations, mainly when they occur at relapse. In the absence of standardized treatment guidelines, individualizing therapy and accessing novel strategies may be essential for improving patient survival.

 

摘要翻译: 

**背景/目的:** 多发性骨髓瘤髓外受累代表一种侵袭性表型,与生存期缩短和不良预后相关。胸部表现较为罕见,文献中对其特征描述不足。 **方法:** 我们在Fundeni临床研究所进行了一项回顾性单中心研究,纳入2010年2月至2025年2月期间诊断为多发性骨髓瘤的患者。研究队列包括34例存在肺实质浸润、胸膜受累或骨髓瘤性胸腔积液的患者。诊断通过影像学检查(计算机断层扫描或磁共振成像)、细胞学检查、免疫分型以及可行情况下的组织病理学确认相结合的方式进行。 **结果:** 在总计2012例多发性骨髓瘤患者中,胸膜-肺髓外受累的发生率为1.6%。诊断时的中位年龄为58岁。26.5%的病例在初次诊断时即存在胸膜-肺疾病,而73.5%的病例在复发时出现。最常见的表现是胸膜受累合并骨髓瘤性积液(70.6%)。不良预后标志物包括β2-微球蛋白水平升高(超过80%的病例)以及约50%的病例乳酸脱氢酶(LDH)升高。研究还发现了细胞遗传学异常,如del(17p)、t(4;14)、t(14;16)、t(11;14)和1q增益。从诊断胸膜-肺髓外疾病起,中位总生存期(OS)为16个月,2年生存率为25%。无患者生存超过5年。中位无进展生存期(PFS)为9个月。 **结论:** 我们的研究结果证实了这些疾病表现的侵袭性临床病程和不良预后,尤其是在复发时出现的情况。由于缺乏标准化的治疗指南,个体化治疗和采用新策略对于改善患者生存可能至关重要。

 

 

原文链接:

Pleuro-Pulmonary Extramedullary Plasmacytomas in Multiple Myeloma: A 15-Year Experience from a Tertiary Center

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