[1]李延娟,张 澍,桑丽娜,等.症状性华氏巨球蛋白血症患者的临床特征及预后影响因素分析[J].郑州大学学报(医学版),2019,(04):627-630.[doi:10.13705/j.issn.1671-6825.2018.11.056]
 LI Yanjuan,ZHANG Shu,SANG Lina,et al.Clinical features and prognostic factors of patients with symptomatic Waldenstrm's macroglobulinemia[J].JOURNAL OF ZHENGZHOU UNIVERSITY(MEDICAL SCIENCES),2019,(04):627-630.[doi:10.13705/j.issn.1671-6825.2018.11.056]
点击复制

症状性华氏巨球蛋白血症患者的临床特征及预后影响因素分析()
分享到:

《郑州大学学报(医学版)》[ISSN:1671-6825/CN:41-1340/R]

卷:
期数:
2019年04期
页码:
627-630
栏目:
应用研究
出版日期:
2019-07-20

文章信息/Info

Title:
Clinical features and prognostic factors of patients with symptomatic Waldenström's macroglobulinemia
作者:
李延娟张 澍桑丽娜汤 平陈 黎陈丹丹孙 玲
郑州大学第一附属医院血液科 郑州 450052
Author(s):
LI Yanjuan ZHANG Shu SANG Lina TANG Ping CHEN Li CHEN Dandan SUN Ling
Department of Hematology,the First Affiliated Hospital,Zhengzhou University,Zhengzhou 450052
关键词:
华氏巨球蛋白血症 淋巴瘤 临床特点
Keywords:
Waldenström's macroglobulinemia lymphoma clinical feature
分类号:
R733
DOI:
10.13705/j.issn.1671-6825.2018.11.056
摘要:
目的:探讨症状性华氏巨球蛋白血症(WM)的临床特点及预后因素。方法:收集郑州大学第一附属医院确诊的60例症状性WM患者的临床资料,分析患者临床特征,采用单因素分析和Cox回归模型分析影响患者生存时间的因素。结果:60例症状性WM患者中33(55.0%)例表现为乏力、头晕等贫血相关症状,38(63.3%)例有淋巴结肿大,52(86.7%)例为IgMκ型。55例行骨髓免疫表型检测,其中50(90.9%)例免疫分型为CD19+、CD20+、CD10-、CD5-、CD79+、FMC7+、CD23-。18例患者中14(77.8%)例MYD88L265P突变阳性。单因素分析结果显示血浆白蛋白水平、乳酸脱氢酶水平、IgA定量、IgM定量、化疗方案均与生存时间有关(P<0.05)。Cox多因素分析显示血浆白蛋白水平是影响症状性WM患者生存时间的独立危险因素,HR(95%CI)为8.632(1.912~38.983)。结论:症状性WM的主要临床表现为贫血相关症状,常见体征为淋巴结肿大,IgMκ型多见。MYD88基因突变率较高。血浆白蛋白水平为影响其预后的独立危险因素。
Abstract:
Aim:To investigate the clinical features and prognostic factors of patients with symptomatic Waldenström's macroglobulinemia(WM).Methods:The clinical data of 60 symptomatic WM patients were collected. The clinical features of the patients were analyzed. Univariate analysis and Cox regression analysis were used to analyze the factors affecting the survival time.Results:Among the 60 patients with symptomatic WM, 33(55.0%)showed anemia-related symptoms such as fatigue and dizziness, 38(63.3%)had lymphadenopathy, and 52(86.7%)had IgMκ style. Out of the 55 patients, 50(90.9%)were immunosuppressed for CD19+, CD20+, CD10-, CD5-, CD79+, FMC7+, and CD23-. Out of the 18 patients, 14(77.8%)had positive MYD88L265P mutations. Univariate analysis showed that albumin level, lactate dehydrogenase level, IgA quantification, IgM quantification, and chemotherapy regimens were associated with survival time(P<0.05). Cox regression analysis showed that albumin level was an independent risk factor for survival time in symptomatic WM patients, with HR(95%CI)of 8.632(1.912-38.983).Conclusion:The main clinical manifestations of symptomatic WM are anemia-related symptoms.Lymphadenopathy is a common clinical sign, and IgMκ is more common style. The mutation rate of MYD88 gene is high. Plasma albumin level is an independent risk factor that influences the prognosis.

参考文献/References:

[1] SWERDLOW SH, CAMPO E, PILERI SA, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms[J].Blood, 2016, 127(20): 2375
[2] GERTZ MA.Waldenström macroglobulinemia: 2017 update on diagnosis, risk stratification and management[J].Am J Hematol,2017,92(2):209
[3] 中国抗癌协会血液肿瘤专业委员会,中华医学会血液学分会白血病淋巴瘤学组,中国抗淋巴瘤联盟.淋巴浆细胞淋巴瘤/华氏巨球蛋白血症诊断与治疗中国专家共识(2016年版)[J].中华血液学杂志,2016,37(9):729
[4] OWEN RG,TREON SP,AL-KATIB A,et al.Clinicopathological definition of Waldenstrom's maeroglobulinemia:consensus panel recommendations from the Second International Workshop on Waldenstrom's Macroglobulinemia[J].Semin Oncol,2003,30(2):110
[5] MOREL P,DUHAMEL A,GOBBI P,et al.International prognostic scoring system for Waldenstrom macroglobulinemia[J].Blood,2009,113(18):4163
[6] CASTILLO JJ, D'SA S, LUNN MP,et al. Central nervous system involvement by Waldenström macroglobulinaemia(Bing-Neel syndrome): a multi-institutional retrospective study[J]. Br J Haematol,2016,172(5):709
[7] 曹欣欣,孟琦,蔡昊,等.华氏巨球蛋白血症患者的临床特征、MYD88L265P、CXCR4WHIM突变和预后:单中心93例回顾性分析[J].中华血液学杂志,2017,38(6):494
[8] WANG H,CHEN Y,LI F, et al.Temporal and geographic variations of Waldenstrom macroglobulinemia incidence:a large population-based study[J].Cancer,2012,118(15):3793
[9] DIMOPOULOS MA, PANAYIOTIDIS P, MOULOPOULOS LA, et al. Waldenström's macroglobulinemia: clinical features, complications, and management[J]. J Clin Oncol, 2000,18(1):214
[10] YI S,CUI R,LI Z,et al. Distinct characteristics and new prognostic scoring system for Chinese patients with Waldenström macroglobulinemia[J]. Chin Med J(Engl), 2014,127(12):2327
[11]CHAKRABORTY R,NOVAK AJ,ANSELL SM,et al.First report of MYD88 L265P somatic mutation in IgM-associated light-chain amyloidosis[J].Blood,2016,127(23):2936
[12]TREON SP, CAO Y, XU L,et al.Somatic mutations in MYD88 and CXCR4 are determinants of clinical presentation and overall survival in Waldenstrom macroglobulinemia[J]. Blood, 2014,123(18):2791
[13]BUSTOROS M, SKLAVENITIS -PISTOFIDIS R, KAPOOR P,et al.Progression risk stratification of asymptomatic Waldenström macroglobulinemia[J].J Clin Oncol,2019,37(16):1403
[14]BUSKE C,HOSTER E,DREYLING M,et al.The addition of rituximab to front-line therapy with CHOP(R-CHOP)results in a higher response rate and longer time to treatment failure in patients with lymphoplasmacytic lymphoma: results of a randomized trial of the German Low-Grade Lymphoma Study Group(GLSG)[J]. Leukemia,2009,23(1):153
[15]LEBLOND V,KASTRITIS E,ADVANI R,et al.Treatment recommendations from the Eighth International Workshop on Waldenstrom's Macroglobulinemia[J]. Blood,2016,128(10):1321
[16]TREON SP, IOAKIMIDIS L, SOUMERAI JD, et al. Primary therapy of Waldenström macroglobulinemia with bortezomib,dexamethasone, and rituximab: WMCTG clinical trial 05-180[J].J Clin Oncol, 2009,27(23):3830
[17]FOUQUET G,GUIDEZ S,PETILLON MO,et al.Lenalidomide is safe and active in Waldenström macroglobulinemia[J].Am J Hematol,2015,90(11):1055

相似文献/References:

[1]姚 珂),杜 正),刘 瀛),等.基于超高效液相-质谱联用技术的淋巴瘤患者血浆代谢组学分析*[J].郑州大学学报(医学版),2017,(01):20.[doi:10.13705/j.issn.1671-6825.2017.01.006]
 YAO Ke),DU Zheng),LIU Ying),et al.Ultra high performance liquid chromatography-mass spectrometry based plasma metabonomics for patients with lymphoma[J].JOURNAL OF ZHENGZHOU UNIVERSITY(MEDICAL SCIENCES),2017,(04):20.[doi:10.13705/j.issn.1671-6825.2017.01.006]

备注/Memo

备注/Memo:
【基金项目】国家自然科学基金资助项目(81400108)
【作者简介】孙玲,通信作者,女,1957年12月生,博士,博士研究生导师,教授,主任医师,研究方向:血液病基础与临床,E-mail:sunling6686@126.com
更新日期/Last Update: 2019-07-20