多发性骨髓瘤伴中枢侵犯临床特征及预后分析

doi:10.3969/j.issn.1006-7795.2023.01.024

摘要/Abstract

摘要： 目的探讨多发性骨髓瘤(multiple myeloma, MM)中枢侵犯患者的临床病理特点及预后生存情况。方法本研究共筛查1 529例MM患者,其中18例伴中枢侵犯,分析其MM及中枢病变的临床病理学特征、生存情况及预后相关因素。结果 MM中枢侵犯发生率1.2%,平均年龄(54.5±11.0)岁,平均Ki-67指数为50.9%±24.2%,荧光原位杂交(fluorescence in situ hybridization, FISH)细胞遗传学高危占43.8%,8例患者(44.4%)中枢侵犯发生于MM初诊时,10例(55.6%)发生于MM进展时,自MM诊断至发生中枢侵犯的中位时间为23.5(14.3,36.0)个月。发生中枢侵犯后中位生存时间为18.0(95%CI: 7.3~28.7)个月,单因素分析,FISH遗传学高危者中位生存时间较标危者更短(7.0个月vs 24.0个月,P=0.007)、Ki-67指数＞50%者生存时间更短(13.0个月vs 29.5个月,P=0.042)。结论 MM初诊及进展时应常规筛查中枢影像学,MM伴中枢侵犯具有侵袭性生物学特点,预后差,FISH遗传学高危及Ki-67指数大于50%为其可能的不良预后因素。

关键词: 多发性骨髓瘤, 中枢神经系统, 临床特征, 预后

Abstract: Objective To investigate the clinicopathological features and prognostic survival of multiple myeloma (MM) with central nervous system (CNS) involvement. Methods Among 1 529 MM patients reviewed, totally 18 MM patients with CNS involvement were included in this retrospective study. Both clinical and prognostic characteristics of MM and the involved intracranial site were analyzed. Results CNS involvement occurred in 1.2% of MM patients. The average age was (54.5±11.0) years. The fluorescence in situ hybridization (FISH)detection suggested that 43.8% of patients with CNS MM had high-risk cytogenetic abnormality with mean Ki-67 proliferation index of 50.9%±24.2%. Eight patients (44.4%) were diagnosed with CNS involvement at an initial MM diagnosis and 10 (55.6%) at relapse/progression. The median time from MM diagnosis to CNS MM diagnosis was 23.5(14.3,36.0)months. The median overall survival (OS) from the onset of CNS involvement for the entire group was 18.0(95%CI: 7.3－28.7)months. The univariate analysis indicated that the patients with high-risk cytogenetic abnormality by FISH (7.0 months vs 24.0 months, P=0.007) and those with a Ki-67 proliferation index of ＞50% (13.0 months vs 29.5 months, P=0.042) had shorter OS. Conclusion A CNS imaging screening should be considered at initial diagnosis and relapse/progression of MM. Patients with CNS MM had invasive biological characteristics and the prognosis was generally poor. High-risk cytogenetic abnormality by FISH and a Ki-67 proliferation index of ＞50% were associated with worse OS.

Key words: multiple myeloma, central nervous system, clincical characteristics, prognosis

中图分类号:

贾静, 陈文明, 李燕郴. 多发性骨髓瘤伴中枢侵犯临床特征及预后分析[J]. 首都医科大学学报, 2023, 44(1): 161-166.

Jia Jing, Chen Wenming, Li Yanchen. Clinical characteristics and prognostic analysis of multiple myeloma involving central nervous system[J]. Journal of Capital Medical University, 2023, 44(1): 161-166.

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