發(fā)布時間：2018-07-13 10:24

【摘要】：免疫性血小板減少癥(immune thrombocytopenia,ITP)是兒童最常見的出血性疾病之一,兒童期多為原發(fā)性,發(fā)病率約為(4~5)/10萬[1]。多數(shù)學者認為ITP是由于機體產生血小板自身抗體,造成血小板破壞過多;和(或)骨髓巨核細胞分化成熟障礙,使血小板生成減少,從而出現(xiàn)皮膚黏膜、臟器出血。其發(fā)病機制涉及體液免疫、細胞免疫、免疫調節(jié)網絡異常和遺傳基質。ITP以不同程度的出血為主要臨床表現(xiàn),輕癥者僅有皮膚黏膜的出血,重癥ITP可致死亡。ITP在兒童中多為自限性,80%的ITP患兒1年內血小板(PLT)數(shù)可恢復正常,部分患兒可出現(xiàn)病情遷延反復或轉為慢性,10%-20%發(fā)展為慢性ITP,約30%的慢性ITP患兒仍在確診數(shù)月或數(shù)年后恢復[2]。鑒于兒童ITP多為自限性過程,治療的目的主要為防止嚴重的出血,而不是提高血小板(PLT)數(shù)至正常值。為進一步研究ITP患兒各型臨床特征及療效等方面的特點,本研究對2012年7月—2015年6月鄭大三附院兒童血液科住院治療的280例ITP患兒進行回顧性分析。目的探討兒童免疫性血小板減少癥(ITP)的臨床特征及同時分析三種不同治療方法療效對比。材料與方法回顧性分析2012年7月-2015年6月鄭大三附院住院治療的280例資料完整并有隨訪記錄的ITP患兒臨床資料,統(tǒng)計各型ITP臨床特征等相關數(shù)據,并對單用激素、激素聯(lián)合丙種球蛋白、單用丙種球蛋白3種不同治療方案的治療效果進行分析。結果采用SPSS 21.0軟件處理,計數(shù)資料(%)表示,率的比較采用卡方檢驗(χ2),等級資料采用秩和檢驗,P0.05表示差異具有統(tǒng)計學意義。結果(1)本研究中280例ITP患兒中新診斷型ITP179例(63.93%),持續(xù)性69例(24.64%),慢性32例(11.43%);男168例,女112例,比例為1.5:1。(2)起病誘因明確者164例(58.57%),發(fā)病季節(jié)以冬春季節(jié)為主;發(fā)病年齡以嬰幼兒為主,各年齡階段男女比率無顯著性差異(P0.05),發(fā)病前1個月內8例ITP患兒有疫苗接種史(4.88%)。(3)本研究中280例ITP患兒的血小板呈不同程度減少;新診斷、持續(xù)性ITP患兒血小板以重度、極重度減少為主;慢性型則表現(xiàn)為中度或重度減少為主,三種分型ITP血小板減少程度差異有統(tǒng)計學意義(P0.001);另外,275例患兒(98.21%)有皮膚黏膜出血表現(xiàn),其中合并鼻衄26例,牙齦出血18例,消化道出血12例,便血2例,未見顱內出血表現(xiàn)。(4)本研究中242例患兒進行了骨髓檢查:細胞形態(tài)學均為增生活躍或增生明顯活躍,粒紅比正常,其中巨核細胞數(shù)增多204例(84.30%),巨核細胞數(shù)正常38例(15.70%)。(5)新診斷、持續(xù)性、慢性ITP采用激素+丙種球蛋白(IVIG)組聯(lián)合治療總有效率分別為90.70%、72.72%、66.67%,差異具有統(tǒng)計學意義(P0.05)。結論兒童ITP以新診斷型多見,持續(xù)性、慢性型少見;發(fā)病年齡以嬰幼兒為主;半數(shù)以上患兒發(fā)病前有前驅感染史;新診斷型ITP起病前多有誘因。激素聯(lián)合丙種球蛋白對新診斷型ITP療效最明顯。
[Abstract]:Immune thrombocytopenia is one of the most common hemorrhagic diseases in children. Most scholars believe that ITP is due to the production of platelet autoantibodies, resulting in excessive platelet destruction; and / or bone marrow megakaryocyte differentiation and maturation obstacle, platelet formation decreased, resulting in skin mucosa, organ bleeding. Its pathogenesis involves humoral immunity, cellular immunity, immune regulatory network abnormalities and genetic matrix. ITP with different degrees of bleeding as the main clinical manifestations, mild patients only bleeding skin and mucosa, Severe ITP can cause death. The number of platelet (PLT) in 80% of the children with ITP who are mostly self-limited can return to normal within one year. Some children may develop chronic ITPs from 10% to 20%, and about 30% of children with chronic ITP can recover after months or years of diagnosis. In view of the self-limited process of ITP in children, the purpose of treatment is to prevent severe bleeding rather than to increase the number of platelets (PLT) to normal. In order to further study the clinical characteristics and curative effects of various types of children with ITP, 280 cases of children with ITP who were hospitalized in Department of Hematology from July 2012 to June 2015 were retrospectively analyzed. Objective to investigate the clinical features of immune thrombocytopenia (ITP) in children and to compare the efficacy of three different treatments. Materials and methods the clinical data of 280 hospitalized children with ITP from July 2012 to June 2015 were analyzed retrospectively, and the clinical characteristics of each type of ITP were analyzed. The therapeutic effects of hormone combined with gamma globulin and gamma globulin alone were analyzed. Results SPSS21.0 software was used to process the data. The counting data (%) were used. The rate was compared by chi-square test (蠂 ~ 2), and the rank sum test was used to show the difference was statistically significant. Results (1) among 280 cases of ITP, 179 cases (63.93%) were newly diagnosed ITP, 69 cases (24.64%) were persistent, 32 cases (11.43%) were chronic, 168 cases were males and 112 cases were females. (2) 164 cases (58.57%) had definite inducement, the season of onset was mainly winter and spring, and the age was mainly infantile. There was no significant difference in the ratio of male and female at different age stages (P0.05). In the first month before the onset of the disease, there was a history of vaccination (4.88%). (3). In this study, the platelets of 280 children with ITP decreased in varying degrees. In the chronic type, there was significant difference in the degree of thrombocytopenia among the three types of ITP (P0.001), and in 275 cases (98.21%) of the children, there were skin and mucosal bleeding, among which 26 cases were complicated with epistaxis. There were 18 cases of gingival bleeding, 12 cases of gastrointestinal bleeding, 2 cases of hematochezia without intracranial hemorrhage. (4) in this study, 242 children were examined by bone marrow examination: the cell morphology was active or hyperplastic, and the granulocyte ratio was normal. The number of megakaryocytes was increased in 204 cases (84.30%), the number of megakaryocytes was normal in 38 cases (15.70%), the total effective rate was 90.70% (84.30%). The total effective rate was 90.70% (84.30%) in the). (group. The difference was statistically significant (P0.05). Conclusion the newly diagnosed ITP is more common, persistent, chronic type is rare, the age of onset is mainly infantile, more than half of the children have a history of pre-infection before onset, and there are more inducements before the onset of newly diagnosed ITP. Hormone combined with gamma globulin is the most effective in the treatment of newly diagnosed ITP.
【學位授予單位】：鄭州大學
【學位級別】：碩士
【學位授予年份】：2017
【分類號】：R725.5

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