原发性浆细胞白血病27例临床特征和预后分析  

Clinical characteristics and prognosis of 27 patients with primary plasma cell leukemia

作　　者：庞海丽 张丹凤 樊文娟[1] 郭荣群 姜中兴[1] PANG Haili;ZHANG Danfeng;FAN Wenjuan;GUO Rongqun;JIANG Zhongcing(Department of Hematology,the First Affiliated Hospital of Zhengzhou University,Zhengzhou,450000,China)

机构地区：[1]郑州大学第一附属医院血液科,郑州450000

出　　处：《临床血液学杂志》2023年第7期514-519,共6页Journal of Clinical Hematology

基　　金：国家自然科学基金(No:82100219)。

摘　　要：目的:探讨原发性浆细胞白血病(primary plasma cell leukemia,pPCL)患者的临床特征及预后分析。方法:选取在郑州大学第一附属医院2018年5月—2022年5月就诊的pPCL患者,收集患者的临床特征、细胞免疫表型、FISH检测、NGS基因检测、治疗和预后情况,结合收集的资料进行单因素和多因素生存分析。结果:27例pPCL患者中位发病年龄为58(29~82)岁,男13例(48.1%)。单因素生存分析结果显示,年龄、是否化疗、外周血中浆细胞(PBPCs)比例、CD27表达、血钙>2.65 mmol/L、β2-MG水平影响pPCL患者预后(P<0.05);多因素生存分析显示,是否接受化疗、PBPCs比例、CD27表达、血钙>2.65 mmol/L独立影响患者的总生存(P<0.05)。结论:是否接受化疗、PBPCs、CD27表达、血钙>2.65 mmol/L独立影响患者的总生存,即提示初诊时患者未接受化疗、PBPCs水平越高、伴有CD27表达、血钙>2.65 mmol/L,患者的疾病进展和死亡的风险越高。Objective:To explore the clinical features and prognosis of patients with primary plasma cell leukemia(pPCL).Methods:The pPCL patients who visited the First Affiliated Hospital of Zhengzhou University from May 2018 to May 2022 were selected,and the clinical characteristics,cellular immunophenotype,FISH detection,NGS gene detection,treatment and prognosis of the patients were collected,and univariate and multi-factor survival analysis was carried out based on the collected data.Results:The median age of onset was 58 years in 27 patients with pPCL and 13 cases(48.1%)were male.The results of univariate survival analysis showed that age,whether to receive chemotherapy,the proportion of PBPCs,the expression of CD27,serum calcium>2.65 mmol/L,β2-MG affected the prognosis of patients with pPCL(P<0.05).Multivariate survival analysis showed that only whether to receive chemotherapy,the proportion of PBPCs,the expression of CD27 and serum calcium>2.65 mmol/L independently affected the overall survival of patients(P<0.05).Conclusion:Whether to receive chemotherapy,the proportion of PBPCs,the expression of CD27 and serum calcium>2.65 mmol/L independently affect the patient's overall survival.That is,when the primary-care patient does not receive chemotherapy,the higher the level of PBPCs,accompanied by CD27 expression and serum calcium>2.65 mmol/L,the higher risk of disease progression and death.

关 键 词：原发性浆细胞白血病 临床特征 免疫表型 细胞遗传学 治疗 预后 

分 类 号：R733.7[医药卫生—肿瘤]