The clinical characteristics, laboratory results, response to treatment, and prognosis of 46 macrofocal multiple myeloma(MFMM) patients at our center from January 2013 to December 2019 were analyzed retrospectively. The other 92 patients were selected as matched-controls based on diagnostic period and treatment. Among the 1 137 MM patients, 46 patients met the definition criteria of MFMM (4.0%), with median age 56 years, which was not statistically different from whole MM population (P=0.066). According to the international staging system (ISS) and Revised ISS, the proportion of patients with advanced stage in MFMM group was less common than that of controls (P<0.05). More plasmacytomas in MFMM patients were presented (43.5% vs. 18.5%, P<0.05). Regarding cytogenetic abnormalities, there were minor patients manifesting high-risk features in MFMM group (15.8% vs. 32.2%, P=0.058). Translocation(11;14) could be detected in 32.4% MFMM patients and 9.4% typical myeloma patients (P<0.05). The treatment regimens were comparable. As to the best response of treatment, the complete response (CR) rate in MFMM group was significantly higher than that of controls (78.3% vs. 60.9%, P<0.05). The median follow-up time was 37.9 months. The median progression-free survival in MFMM and control groups were 77.5 vs. 39.8 months, respectively (P<0.05). The overall survival (OS) of MFMM patients was significantly longer (not reached vs. 68.2 months, P<0.05).
回顾性分析2013年1月至2019年12月就诊于中国医学科学院血液病医院的46例巨灶型骨髓瘤（MFMM）患者临床特征、化验及检查结果、具体治疗方案、疗效及生存等临床资料，并按照诊断时间、治疗方案匹配，选择92例经典多发性骨髓瘤（MM）患者作为对照组。在1 137例MM患者中，符合MFMM诊断标准患者46例（4.0%），中位年龄56岁，与经典MM患者差异无统计学意义（P=0.066）；MFMM组41例（89.1%）患者为ISS分期Ⅰ、Ⅱ期，仅5例（10.9%）患者为ISS Ⅲ期，而对照组49例（53.3%）患者为ISS Ⅲ期（P<0.05）；所有MFMM患者均为R-ISS分期Ⅰ、Ⅱ期。MFMM组20例患者初诊时可见髓外浆细胞瘤，比例明显高于对照组（43.5% 比 18.5%，P<0.05）。MFMM组较少出现高危遗传学异常，比例低于对照组（15.8% 比 32.2%，P=0.058）；MFMM组患者t（11；14）发生率明显高于对照组（32.4% 比 9.4%，P<0.05）。MFMM组与对照组患者诱导治疗方案、自体移植率相似，MFMM组中最佳疗效达CR患者比例明显高于对照组（78.3% 比 60.9%，P<0.05）；截至2021年7月，中位随访时间37.9个月，MFMM组患者中位无进展生存期较对照组明显延长（77.5个月 比 39.8个月，P<0.05），总生存期也显著优于对照组（未达到 比 68.2个月，P<0.05）。骨髓浆细胞数量、不同新药治疗对MFMM患者生存结局无影响。.