文章：

多发性骨髓瘤诊断时及首次复发时的骨旁与髓外浆细胞瘤：某学术机构50年经验

Paraskeletal and extramedullary plasmacytomas in multiple myeloma at diagnosis and at first relapse: 50-years of experience from an academic institution

原文发布日期：2022-09-16

DOI: 10.1038/s41408-022-00730-5

类型: Article

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英文摘要：

From January 1970 to December 2018, 1304 patients were diagnosed with multiple myeloma (MM) at our institution and 256 (19.6%) had plasmacytomas (Ps) (paraskeletal –PPs- 17.6%, extramedullary –EMPs-1.9%). Patients with Ps had lower serum M-protein and less advanced ISS stage than those without. At first relapse, 192 out of 967 patients (19.8%) developed Ps (PPs 14.6%, EMPs 5.1%). The only factor associated with Ps at relapse was the presence of Ps at diagnosis (46% vs 13%, p < 0.00001) with no impact with exposure to novel drugs or previous autologous stem-cell transplantation (ASCT). The median overall survival (OS) was 45, 44 and 20 months for patients without Ps, PPs and EMPs, respectively (p = 0.013). Patients with PPs who underwent ASCT had similar OS than those without Ps (98 vs. 113 months) and significantly longer than those with EMPs (98 vs 47 months, p = 0.006). In patients non-eligible for ASCT the presence of PPs or EMPs was associated with shorter OS compared with patients without Ps (32 vs. 24 vs. 6 months, p = 0.009). In the relapsed setting, a significant survival benefit was observed beyond the year 2000, but still with significant differences among patients without Ps, PPs and EMPs (37 vs 22 vs 16 months, p = 0.003). Importantly, rescue therapy with combinations of proteasome-inhibitors plus immunomodulatory drugs was associated with prolonged OS from first relapse (over 6 years), even in patients with EMPs.
 

摘要翻译： 

从1970年1月至2018年12月，我院共有1304例患者被诊断为多发性骨髓瘤（MM），其中256例（19.6%）伴有浆细胞瘤（Ps）（旁骨骼浆细胞瘤（PPs）占17.6%，髓外浆细胞瘤（EMPs）占1.9%）。伴有浆细胞瘤的患者血清M蛋白水平较低，ISS分期较不伴有浆细胞瘤的患者更早。在首次复发时，967例患者中有192例（19.8%）出现浆细胞瘤（PPs占14.6%，EMPs占5.1%）。复发时出现浆细胞瘤的唯一相关因素是诊断时已存在浆细胞瘤（46% vs 13%，p < 0.00001），而暴露于新药或既往自体干细胞移植（ASCT）对其无影响。不伴有浆细胞瘤、旁骨骼浆细胞瘤和髓外浆细胞瘤患者的中位总生存期（OS）分别为45、44和20个月（p = 0.013）。接受ASCT的旁骨骼浆细胞瘤患者与不伴有浆细胞瘤患者的总生存期相似（98 vs. 113个月），且显著长于髓外浆细胞瘤患者（98 vs 47个月，p = 0.006）。在不适合ASCT的患者中，伴有旁骨骼浆细胞瘤或髓外浆细胞瘤与不伴有浆细胞瘤的患者相比，总生存期更短（32 vs. 24 vs. 6个月，p = 0.009）。在复发背景下，2000年后观察到显著的生存获益，但不伴有浆细胞瘤、旁骨骼浆细胞瘤和髓外浆细胞瘤患者之间仍存在显著差异（37 vs 22 vs 16个月，p = 0.003）。重要的是，蛋白酶体抑制剂联合免疫调节药物的挽救治疗与首次复发后总生存期延长（超过6年）相关，即使在髓外浆细胞瘤患者中也是如此。

原文链接：

Paraskeletal and extramedullary plasmacytomas in multiple myeloma at diagnosis and at first relapse: 50-years of experience from an academic institution