多发性骨髓瘤中枢神经系统浸润7例临床分析

Multiple myeloma involving central nervous system infiltration:a report of 7 cases

目的探讨多发性骨髓瘤(MM)累及中枢神经系统(CNS)的诊治及预后。方法选取2005年1月至2019年8月于北部战区总医院就诊的381例MM患者中CNS受累者6例,以及就诊于中国医科大学附属盛京医院的MM浸润CNS患者1例,回顾性分析其临床资料,随访时间从确诊MM至死亡。结果北部战区总医院就诊MM患者CNS浸润发生率为1.57%(6/381)。7例患者中,4例累及脑实质,2例累及软脑膜,1例同时累及脑实质和软脑膜;4例乳酸脱氢酶(LDH)水平升高。2例IgG型及1例λ型患者,CNS浸润时M蛋白水平正常且骨髓浆细胞比例不高。脑脊液蛋白水平增高3例;2例骨髓瘤细胞CD56缺失;2例1q21+、2例17p13-、1例t(4;14)、1例13q-。4例初诊CNS浸润患者中,仅1例达到完全缓解后行自体造血干细胞移植,移植后11个月仍持续缓解;其余3例生存时间分别为4、5、11个月。3例病程中CNS浸润患者生存时间分别为3、4、8个月。结论MM累及CNS较为少见,预后多不良,生存期较短,在治疗上尚缺乏有效的干预手段。

Objective To investigate the diagnosis,treatment and prognosis of multiple myeloma(MM)involving central nervous system(CNS)infiltration.Methods Among 381 MM patients admitted to General Hospital of Northern Theater Command from January 2005 to August 2019,clinical data of 6 patients with CNS infiltration and 1 MM patient with CNS infiltration admitted to Shengjing Hospital affiliated to China Medical University were selected for retrospective analysis.Follow-up lasted from diagnosis to death.Results The incidence of CNS infiltration in MM patients treated in General Hospital of Northern Theater Command was 1.57%(6/381).Among the 7 patients with CNS,4 cases involved brain parenchyma,2 cases involved leptomeninges,1 case involved both brain parenchyma and pia mater,and 4 cases had elevated level of lactate dehydrogenase(LDH).In 2 cases of IgG type and 1 case ofλtype,the level of M protein was normal and the level of bone marrow plasma cells was not high during CNS infiltration.The protein level of cerebrospinal fluid increased in 3 cases,CD56 deletion in myeloma cells in 2 cases,1q21+in 2 cases,17p13-in 2 cases,t(4;14)in 1 case and 13q-in 1 case.Among the 4 patients with newly diagnosed CNS infiltration,only 1 patient received autologous hematopoietic stem cell transplantation after complete remission and remained in remission 11 months after transplantation,while the survival time of the other 3 patients was 4,5 and 11 months respectively.The survival time of 3 patients with CNS infiltration was 3,4 and 8 months,respectively.Conclusion MM involving CNS is rare,and its prognosis is poor,which still has no effective intervention in the treatment.