本文選題：骨髓增生異常綜合征　切入點(diǎn)：分型　出處：《山東大學(xué)》2017年碩士論文　論文類型：學(xué)位論文

【摘要】：目的:通過對(duì)骨髓增生異常綜合征患者臨床資料的分析,同時(shí)與國內(nèi)外臨床資料進(jìn)行對(duì)比,探討本地區(qū)骨髓增生異常綜合征發(fā)病的臨床特征,為其早期診斷和有效治療提供依據(jù)。材料和方法:收集2012年1月至2016年10月在山東省立醫(yī)院血液科就診的初治骨髓增生異常綜合征患者239例,所有病例均根據(jù)骨髓及外周血涂片Wright-Giemsa染色后的形態(tài)學(xué)及染色體檢查結(jié)果,按照世界衛(wèi)生組織分類標(biāo)準(zhǔn)確診。回顧性分析其性別、年齡、臨床表現(xiàn)、分型、預(yù)后分組、血常規(guī)、貧血三項(xiàng)、血清促紅細(xì)胞生成素水平以及骨髓穿刺、活檢和染色體、熒光原位雜交檢查等。應(yīng)用SPSS統(tǒng)計(jì)軟件進(jìn)行數(shù)據(jù)分析,p0.05有統(tǒng)計(jì)學(xué)意義。結(jié)果:在239例骨髓增生異常綜合征患者中,男性患者136例,女性患者103例,男女發(fā)病比例為1.32:1,平均發(fā)病年齡為55.0歲。所有患者就診時(shí)均有一系血細(xì)胞低于正常參考值,三系均低患者最常見(56.49%)。患者初診時(shí)的臨床癥狀多為貧血導(dǎo)致的頭痛頭暈、面黃乏力等。所有患者均行染色體檢查,染色體異常檢出率為38.08%。按照世界衛(wèi)生組織2008年分型建議,在這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-綜合征患者1例。不同MDS分型患者與年齡、白細(xì)胞計(jì)數(shù)、紅細(xì)胞計(jì)數(shù)、血紅蛋白水平、血小板計(jì)數(shù)、中性粒細(xì)胞計(jì)數(shù)、骨髓增生活躍度、染色體異常比例、活檢標(biāo)本骨髓增生活躍度、FISH異常比例等相關(guān)指標(biāo)差異無統(tǒng)計(jì)學(xué)意義。按照IPSS-R標(biāo)準(zhǔn)對(duì)患者進(jìn)行預(yù)后分組,沒有極低�；颊�,低�；颊�47例,中危患者81例,高�；颊�63例,極高危患者48例。不同MDS預(yù)后分組患者與年齡、活檢標(biāo)本骨髓增生活躍度、FISH異常比例差異無統(tǒng)計(jì)學(xué)意義,與白細(xì)胞計(jì)數(shù)、紅細(xì)胞計(jì)數(shù)、血紅蛋白水平、血小板計(jì)數(shù)、中性粒細(xì)胞計(jì)數(shù)、骨髓增生活躍度、染色體異常比例差異有統(tǒng)計(jì)學(xué)意義。患者的預(yù)后分組越高危,染色體異常的檢出比例越高。對(duì)骨髓增生異常綜合征患者采取個(gè)體化綜合治療措施,主要包括抗感染、成分輸血、刺激造血、祛鐵治療、免疫調(diào)節(jié)治療和免疫抑制治療,以及去甲基化藥物地西他濱、化療以及造血干細(xì)胞移植等高強(qiáng)度治療。239例患者中共有57例患者接受高強(qiáng)度治療(23.85%),其中6例患者接受異基因造血干細(xì)胞移植。在造血干細(xì)胞移植前接受地西他濱橋接治療有可能提高移植的療效,這一治療方案值得進(jìn)一步研究。結(jié)論:骨髓增生異常綜合征是一種異質(zhì)性克隆性造血干細(xì)胞疾病,老年患者多見,無特異性臨床表現(xiàn),主要為貧血、出血、感染,貧血癥狀是促成患者就診的首要原因。完善患者臨床檢查及骨髓相關(guān)檢查對(duì)明確診斷、分型、預(yù)后分組等有重要意義。不同MDS分型患者與年齡、白細(xì)胞計(jì)數(shù)、紅細(xì)胞計(jì)數(shù)、血紅蛋白水平、血小板計(jì)數(shù)、中性粒細(xì)胞計(jì)數(shù)、骨髓增生活躍度、染色體異常比例、活檢標(biāo)本骨髓增生活躍度、FISH異常比例等相關(guān)指標(biāo)差異無統(tǒng)計(jì)學(xué)意義。按照IPSS-R標(biāo)準(zhǔn)對(duì)患者進(jìn)行預(yù)后分組,不同MDS預(yù)后分組患者與年齡、活檢標(biāo)本骨髓增生活躍度、FISH異常比例差異無統(tǒng)計(jì)學(xué)意義,與白細(xì)胞計(jì)數(shù)、紅細(xì)胞計(jì)數(shù)、血紅蛋白水平、血小板計(jì)數(shù)、中性粒細(xì)胞計(jì)數(shù)、骨髓增生活躍度、染色體異常比例差異有統(tǒng)計(jì)學(xué)意義。不同預(yù)后分組的患者,接受相應(yīng)的治療方案,進(jìn)行個(gè)體化治療。異基因造血干細(xì)胞移植仍然是唯一可能治愈MDS的治療手段。移植前行地西他濱橋接治療有望提高移植的療效。
[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.

【學(xué)位授予單位】：山東大學(xué)
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2017
【分類號(hào)】：R551.3

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