出现异常巨核细胞的获得性再生障碍性贫血患者临床特征及转归分析

发布时间：2018-03-24 10:06

本文选题：异常巨核细胞　切入点：再生障碍性贫血　出处：《北京协和医学院》2017年硕士论文

【摘要】：研究目的:观察伴有异常巨核细胞的获得性再生障碍性贫血(Acquired aplastic anemia,AA)患者临床特征、治疗疗效及转归。研究方法:回顾性分析伴有异常巨核细胞的获得性再生障碍性贫血患者临床资料。研究结果:共纳入48例患者,其中男25例,女23例,中位年龄14.5(5-66)岁。中位随访71.99(5.40-175.53)个月。6例为治疗前出现巨核细胞异常,42例治疗后出现巨核细胞异常,巨核细胞首次出现异常的中位时间为35.95(1.27-404.67)个月。48例患者中10例(20.83%)发生疾病转化,其中5例为MDS(10.42%),4例AML(8.33%),1例AA-PHN(2.08%)。中位转化时间66.23(15.73-187.90)个月。结果提示单元核、双元核巨核细胞的比例与疾病转化相关(p0.05)。其中单元核巨核细胞占所有巨核细胞比例为独立预后因素。治疗后疗效与细胞遗传学、G-CSF应用时间、G-CSF应用与否相关。年龄小于等于20岁的患者双核巨核细胞(p=0.04)、单元核巨核细胞(p=0.03)占全部巨核细胞比例明显高于大于20岁患者组。治疗分组、细胞遗传学,G-CSF应用、性别、年龄分组、淋巴样巨核细胞出现、诊断分型均不是影响转化时间的危险因素。研究结论:1.再生障碍性贫血患者中出现异常巨核细胞患者的疾病转化率为20.83%;2.双核巨核细胞、单元核巨核细胞更容易出现在小于20岁的患者人群中,单元核巨核细胞比例与疾病转化相关,但与治疗疗效无明显相关性;3.出现不良细胞遗传学异常以及存在G-CSF应用、G-CSF应用时间长均提示治疗疗效不佳。
[Abstract]:Objective: to observe the clinical characteristics of acquired aplastic anemiaAA-A with abnormal megakaryocyte. Clinical data of patients with acquired aplastic anemia with abnormal megakaryocyte were retrospectively analyzed. Results: a total of 48 patients were included, including 25 males and 23 females. The median age was 14.5 years (5-66). The median follow-up period was 71.995.40-175.53 months. After treatment, 42 patients with megakaryocyte abnormalities appeared megakaryocyte abnormalities, and the median time of the first abnormal megakaryocytes was 35.951.27-404.67 months. Among them, 5 cases were MDS 10.42 and 4 cases were AML8.33 and 1 case was AA-PHN 2.08. The median conversion time was 66.23 卤15.73-187.90 months. The proportion of megakaryocyte in mononuclear cells was associated with disease transformation. The percentage of megakaryocyte in mononuclear cells was an independent prognostic factor. The therapeutic effect after treatment was correlated with the time of G-CSF use in cytogenetics, age and age. The percentage of mononuclear megakaryocyte p0.03) in patients younger than 20 years old was significantly higher than that in patients over 20 years old. Cytogenetic G-CSF application, sex, age group, lymphoid megakaryocytes, Diagnostic typing was not a risk factor for transforming time. Conclusion 1. The disease transformation rate of abnormal megakaryocytes in aplastic anemia patients was 20.833.The binuclear megakaryocyte, Mononuclear megakaryocytes are more likely to appear in patients younger than 20 years of age, and the proportion of mononuclear megakaryocytes is associated with disease transformation. However, there was no significant correlation with the therapeutic efficacy. 3. Abnormal cytogenetic abnormalities and the presence of G-CSF with G-CSF for a long time all indicated that the therapeutic effect was not good.
【学位授予单位】：北京协和医学院
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R556.5

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