骨髓增生异常综合征239例临床病例分析

发布时间：2018-03-10 07:54

本文选题：骨髓增生异常综合征　切入点：分型　出处：《山东大学》2017年硕士论文　论文类型：学位论文

【摘要】：目的:通过对骨髓增生异常综合征患者临床资料的分析,同时与国内外临床资料进行对比,探讨本地区骨髓增生异常综合征发病的临床特征,为其早期诊断和有效治疗提供依据。材料和方法:收集2012年1月至2016年10月在山东省立医院血液科就诊的初治骨髓增生异常综合征患者239例,所有病例均根据骨髓及外周血涂片Wright-Giemsa染色后的形态学及染色体检查结果,按照世界卫生组织分类标准确诊。回顾性分析其性别、年龄、临床表现、分型、预后分组、血常规、贫血三项、血清促红细胞生成素水平以及骨髓穿刺、活检和染色体、荧光原位杂交检查等。应用SPSS统计软件进行数据分析,p0.05有统计学意义。结果:在239例骨髓增生异常综合征患者中,男性患者136例,女性患者103例,男女发病比例为1.32:1,平均发病年龄为55.0岁。所有患者就诊时均有一系血细胞低于正常参考值,三系均低患者最常见(56.49%)。患者初诊时的临床症状多为贫血导致的头痛头晕、面黄乏力等。所有患者均行染色体检查,染色体异常检出率为38.08%。按照世界卫生组织2008年分型建议,在这239例患者中,共有RCUD患者40例,RARS患者15例,RCMD患者88例,RAEB-Ⅰ患者36例,RAEB-Ⅱ患者58例,MDS-U患者1例,5q-综合征患者1例。不同MDS分型患者与年龄、白细胞计数、红细胞计数、血红蛋白水平、血小板计数、中性粒细胞计数、骨髓增生活跃度、染色体异常比例、活检标本骨髓增生活跃度、FISH异常比例等相关指标差异无统计学意义。按照IPSS-R标准对患者进行预后分组,没有极低危患者,低危患者47例,中危患者81例,高危患者63例,极高危患者48例。不同MDS预后分组患者与年龄、活检标本骨髓增生活跃度、FISH异常比例差异无统计学意义,与白细胞计数、红细胞计数、血红蛋白水平、血小板计数、中性粒细胞计数、骨髓增生活跃度、染色体异常比例差异有统计学意义。患者的预后分组越高危,染色体异常的检出比例越高。对骨髓增生异常综合征患者采取个体化综合治疗措施,主要包括抗感染、成分输血、刺激造血、祛铁治疗、免疫调节治疗和免疫抑制治疗,以及去甲基化药物地西他滨、化疗以及造血干细胞移植等高强度治疗。239例患者中共有57例患者接受高强度治疗(23.85%),其中6例患者接受异基因造血干细胞移植。在造血干细胞移植前接受地西他滨桥接治疗有可能提高移植的疗效,这一治疗方案值得进一步研究。结论:骨髓增生异常综合征是一种异质性克隆性造血干细胞疾病,老年患者多见,无特异性临床表现,主要为贫血、出血、感染,贫血症状是促成患者就诊的首要原因。完善患者临床检查及骨髓相关检查对明确诊断、分型、预后分组等有重要意义。不同MDS分型患者与年龄、白细胞计数、红细胞计数、血红蛋白水平、血小板计数、中性粒细胞计数、骨髓增生活跃度、染色体异常比例、活检标本骨髓增生活跃度、FISH异常比例等相关指标差异无统计学意义。按照IPSS-R标准对患者进行预后分组,不同MDS预后分组患者与年龄、活检标本骨髓增生活跃度、FISH异常比例差异无统计学意义,与白细胞计数、红细胞计数、血红蛋白水平、血小板计数、中性粒细胞计数、骨髓增生活跃度、染色体异常比例差异有统计学意义。不同预后分组的患者,接受相应的治疗方案,进行个体化治疗。异基因造血干细胞移植仍然是唯一可能治愈MDS的治疗手段。移植前行地西他滨桥接治疗有望提高移植的疗效。
[Abstract]:Objective: through a comprehensive analysis of the clinical data of patients with syndrome of myelodysplasia, at the same time with the domestic and foreign clinical data were compared, and to investigate the clinical features of local myelodysplastic syndrome, and provide the evidence for early diagnosis and effective treatment. Materials and methods: collected from January 2012 to October 2016 in the Department of Hematology of Shangdong Province-owned Hospital from the initial treatment of bone marrow 239 cases of myelodysplastic syndrome patients, all cases were classified according to the morphology of bone marrow and peripheral blood smears were stained with Wright-Giemsa and the results of chromosome examination, according to the classification standard of WHO accurate diagnosis. A retrospective analysis of the gender, age, clinical manifestation, classification, prognosis group, blood routine, anemia in three, serum erythropoietin level and the bone marrow puncture, biopsy and chromosome fluorescence in situ hybridization test. SPSS statistical software was used for data analysis, P0.05 statistics Significance. Results: in 239 cases of patients with myelodysplastic syndrome, 136 cases of male patients, 103 female patients, male and female incidence ratio was 1.32:1, the average age was 55 years. All patients had a blood cell line was lower than the normal reference value, the three lines were the most common in patients with low (56.49%). The clinical symptoms of patients with anemia caused many headaches and dizziness, pale and fatigue. All patients underwent chromosome examination, the detection rate of the chromosomal abnormality of 38.08%. according to the classification of WHO in 2008, of the 239 patients, a total of 40 RCUD patients, 15 RARS patients, 88 RCMD patients, 36 cases RAEB- I patients, 58 cases of RAEB- patients, 1 MDS-U patients with 5q- syndrome in 1 cases. Different MDS types of patients with age, leukocyte count, erythrocyte count, hemoglobin level, platelet count, neutrophil count, bone marrow proliferation activity of dye The proportion of abnormal body color, increase the activity of bone marrow biopsy specimens, the difference was not statistically significant FISH related indicators abnormal proportion. The prognosis of patients grouped according to the IPSS-R standard, not very low risk patients, 47 cases of low-risk patients, 81 cases of patients, 63 cases of high-risk patients, high-risk patients in 48 cases. Different MDS the prognosis of patients with the age group, bone marrow biopsy specimens increased activity, there was no significant difference with the abnormal ratio of FISH, leukocyte count, erythrocyte count, hemoglobin level, platelet count, neutrophil count, bone marrow proliferation activity, was statistically significant difference. The proportion of abnormal chromosome in patients with the high-risk prognosis group, chromosome the abnormal detection. The higher proportion of myelodysplastic syndrome patients with individualized comprehensive treatment, including anti infection, transfusion of blood components, stimulation of hematopoiesis, iron chelation therapy, immunotherapy and free Treatment of disease suppression, and demethylation drug decitabine, chemotherapy and hematopoietic stem cell transplantation in treatment of high strength.239 patients and 57 patients were treated with high intensity treatment (23.85%), of which 6 patients received allogeneic hematopoietic stem cell transplantation in hematopoietic stem cell transplantation before the West may increase the effect of transplantation of capecitabine bridging treatment, worthy of further research on this treatment. Conclusion: myelodysplastic syndrome is a clonal hematopoietic stem cell disease, more common in older patients, no specific clinical manifestations, mainly for anemia, bleeding, infection, symptoms of anemia is the primary reasons for treatment of patients perfect. Clinical examination and bone marrow examination on the diagnosis, typing, grouping and so on. The clinical significance of different MDS types of patients with age, leukocyte count, erythrocyte count, hemoglobin level, platelet count The number of neutrophil count, bone marrow proliferation activity, the proportion of chromosomal abnormalities, bone marrow biopsy specimens increased activity, the difference was not statistically significant FISH related indicators abnormal proportion. The prognosis of patients grouped according to the IPSS-R standard, the prognosis of MDS patients with different age groups, increase the activity of bone marrow biopsy specimens, no statistically significant the proportion of abnormal FISH difference, and leukocyte count, erythrocyte count, hemoglobin level, platelet count, neutrophil count, bone marrow proliferation activity, statistically significant proportion of abnormal chromosome differences. Different prognosis groups of patients, treatment programs, individualized treatment. Allogeneic hematopoietic stem cell transplantation is still the only possible cure for MDS therapy before transplantation. Decitabine treatment may improve the efficacy of bridging transplantation.

【学位授予单位】：山东大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R551.3

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