CAJ | 학술논문

目的探讨国内极年轻多发性骨髓瘤(MM)患者临床表现及预后.方法回顾性分析单中心35例40岁以下初治MM患者的临床表现及生存情况,并与已发表的西方国家数据进行比较.结果 40岁以下MM患者占同时期初治MM患者的4.6％,与国外数据相比,该部分患者国际分期系统Ⅲ期比例较高,有更高比例的IgD型、贫血、血小板减少及17p13缺失.中位随访17(1～89)个月,35例患者的中位无进展生存(PFS)及总生存(OS)时间分别为13和33个月,截止至随访结束有17例患者死亡,其中8例死于疾病诊断后1年内.单因素分析结果显示,髓外浸润(P=0.031),17p13缺失(P=0.015)及肾功能受损(P=0.000)是影响PFS的不良因素,而17p13缺失(P=0.015)及肾功能受损(P=0.001)是影响OS的不良因素.多因素分析结果显示,肾功能损伤是影响预后的不良因素(PFS:HR=3.953,95％可信区间1.263～12.371,P=0.018;OS:HR=5.769,95％可信区间1.602～20.771,P=0.007).结论国内40岁以下MM患者的临床特征及生存情况与西方国家患者有所不同,因此在临床诊疗过程中应特别重视该亚群患者.
목적 탐토국내겁년경다발성골수류(MM)환자림상표현급예후.방법 회고성분석단중심35례40세이하초치MM환자적림상표현급생존정황,병여이발표적서방국가수거진행비교.결과 40세이하MM환자점동시기초치MM환자적4.6％,여국외수거상비,해부분환자국제분기계통Ⅲ기비례교고,유경고비례적IgD형、빈혈、혈소판감소급17p13결실.중위수방17(1～89)개월,35례환자적중위무진전생존(PFS)급총생존(OS)시간분별위13화33개월,절지지수방결속유17례환자사망,기중8례사우질병진단후1년내.단인소분석결과현시,수외침윤(P=0.031),17p13결실(P=0.015)급신공능수손(P=0.000)시영향PFS적불량인소,이17p13결실(P=0.015)급신공능수손(P=0.001)시영향OS적불량인소.다인소분석결과현시,신공능손상시영향예후적불량인소(PFS:HR=3.953,95％가신구간1.263～12.371,P=0.018;OS:HR=5.769,95％가신구간1.602～20.771,P=0.007).결론 국내40세이하MM환자적림상특정급생존정황여서방국가환자유소불동,인차재림상진료과정중응특별중시해아군환자.
Objective To investigate the clinical characteristics and prognosis of very young patients with multiple myeloma (MM).Methods We retrospectively analyzed the clinical characteristics and outcome of 35 newly diagnosed MM patients 40 years old or younger during a period of 15 years and compared with published data from western countries.Results Our study demonstrated that these very young patients were more likely to be in advanced stage of International staging system,IgD isotype,hemoglobin＜ 100 g/L,decreased platelets and deletion of 17p 13.With a median follow-up of 17months (1-89 months),the median overall survival (OS) of this cohort was 33 months and progression-free survival (PFS) was 13 months,moreover 8 of 17 deaths occurred in the first year after diagnosis.In the univariate analysis,extramedullary infiltration,del (17p13) and renal impairment were associated with reduced PFS (P=0.031,P=0.015,P=000,respectively),while the last two factors also predicted inferior OS (P=0.015,P=0.001),but multivariate analysis showed that only renal impairment independently associated with inferior survival in COX model (PFS:HR=3.953,95％ CI 1.263-12.371,P=0.018;OS:HR=5.769,95％ CI 1.602-20.771,P=0.007).Conclusion Our research showed for the first time that the clinical characteristics and survival of MM patients ≤40 years old in China were different from that in western countries.The special attention should be paid to the patients with their diagnosis and treatment.