儿童免疫性血小板减少症280例临床特征及疗效分析

发布时间：2018-07-13 10:24

【摘要】：免疫性血小板减少症(immune thrombocytopenia,ITP)是儿童最常见的出血性疾病之一,儿童期多为原发性,发病率约为(4~5)/10万[1]。多数学者认为ITP是由于机体产生血小板自身抗体,造成血小板破坏过多;和(或)骨髓巨核细胞分化成熟障碍,使血小板生成减少,从而出现皮肤黏膜、脏器出血。其发病机制涉及体液免疫、细胞免疫、免疫调节网络异常和遗传基质。ITP以不同程度的出血为主要临床表现,轻症者仅有皮肤黏膜的出血,重症ITP可致死亡。ITP在儿童中多为自限性,80%的ITP患儿1年内血小板(PLT)数可恢复正常,部分患儿可出现病情迁延反复或转为慢性,10%-20%发展为慢性ITP,约30%的慢性ITP患儿仍在确诊数月或数年后恢复[2]。鉴于儿童ITP多为自限性过程,治疗的目的主要为防止严重的出血,而不是提高血小板(PLT)数至正常值。为进一步研究ITP患儿各型临床特征及疗效等方面的特点,本研究对2012年7月—2015年6月郑大三附院儿童血液科住院治疗的280例ITP患儿进行回顾性分析。目的探讨儿童免疫性血小板减少症(ITP)的临床特征及同时分析三种不同治疗方法疗效对比。材料与方法回顾性分析2012年7月-2015年6月郑大三附院住院治疗的280例资料完整并有随访记录的ITP患儿临床资料,统计各型ITP临床特征等相关数据,并对单用激素、激素联合丙种球蛋白、单用丙种球蛋白3种不同治疗方案的治疗效果进行分析。结果采用SPSS 21.0软件处理,计数资料(%)表示,率的比较采用卡方检验(χ2),等级资料采用秩和检验,P0.05表示差异具有统计学意义。结果(1)本研究中280例ITP患儿中新诊断型ITP179例(63.93%),持续性69例(24.64%),慢性32例(11.43%);男168例,女112例,比例为1.5:1。(2)起病诱因明确者164例(58.57%),发病季节以冬春季节为主;发病年龄以婴幼儿为主,各年龄阶段男女比率无显著性差异(P0.05),发病前1个月内8例ITP患儿有疫苗接种史(4.88%)。(3)本研究中280例ITP患儿的血小板呈不同程度减少;新诊断、持续性ITP患儿血小板以重度、极重度减少为主;慢性型则表现为中度或重度减少为主,三种分型ITP血小板减少程度差异有统计学意义(P0.001);另外,275例患儿(98.21%)有皮肤黏膜出血表现,其中合并鼻衄26例,牙龈出血18例,消化道出血12例,便血2例,未见颅内出血表现。(4)本研究中242例患儿进行了骨髓检查:细胞形态学均为增生活跃或增生明显活跃,粒红比正常,其中巨核细胞数增多204例(84.30%),巨核细胞数正常38例(15.70%)。(5)新诊断、持续性、慢性ITP采用激素+丙种球蛋白(IVIG)组联合治疗总有效率分别为90.70%、72.72%、66.67%,差异具有统计学意义(P0.05)。结论儿童ITP以新诊断型多见,持续性、慢性型少见;发病年龄以婴幼儿为主;半数以上患儿发病前有前驱感染史;新诊断型ITP起病前多有诱因。激素联合丙种球蛋白对新诊断型ITP疗效最明显。
[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.
【学位授予单位】：郑州大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R725.5

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