摘要
目的 探讨儿童原发性免疫性血小板减少症(ITP)的临床特征,为进一步揭示其发病机制和更精准的分层诊疗提供依据,同时带动提升内蒙古地区ITP规范诊疗.方法 通过多中心回顾性队列研究,分析内蒙古自治区人民医院等内蒙古地区12家医院近6年收治的632例ITP患儿的临床资料,根据年龄分组,通过单因素方差分析得出不同年龄段患儿的临床特点.结果 纳入研究的316例患儿中,不同年龄段的患儿在性别、ICR出血分级、黏膜出血、发病诱因、初诊PLT计数、MPV、ANA、A-ENA、免疫球蛋白、骨髓巨核细胞总数、骨髓粒系比例、不同的治疗方式及治疗后1、3、6、12个月的治疗效果、ITP的分型中具有统计学意义;发病季节、补体水平差异无统计学意义.结论 内蒙古地区儿童ITP发病高峰在4~18岁段,随年龄的增长女性逐渐多于男性;鼻黏膜出血占比、初诊PLT计数及MPV升高、ANA+、A-ENA+、免疫球蛋白水平升高、骨髓巨核细胞总数增高、骨髓粒系比例升高、GC+IVIG治疗、进展为慢性比率较高多分布在4~18岁组;<1岁组有起病诱因占比高于另外两组,且在第1、3、6、12个月的完全缓解率均高于另外2组.
Abstract
Objective To explore the basic clinical characteristics of primary immune thrombocytopenia(ITP)in children,provide a basis for further exploration of pathogenesis and more accurate hierarchical diagnosis and treatment,and promote the improvement of the standardization of diagnosis and treatment in this region.Methods The clinical data of 632 children with ITP treated in 12 hospitals including the People's Hospital of Inner Mongolia Autonomous Region in recent 6 years were collected and divided into 3 groups according to age.The clinical characteristics of the children in different ages were obtained by one-way ANOVA.Results Among the 316 children included in the statistics,There were significant differences in gender,ICR bleeding grade,mucosal bleeding,pathogenesis,PLT count at first diagnosis,MPV,ANA,A-ENA,immunoglobulin,total number of bone marrow megakaryocytes,proportion of bone marrow granules,different treatment methods,treatment effect at 1,3,6 and 12 months after treatment,and ITP classification in children of different ages.The season of onset and complement had no statistical significance.Conclusions In Inner Mongolia,ITP occurred in children aged from 4 to 18 years,and the number of females was higher than that of males.The proportion of nasal mucosal hemorrhage,PLT count and MPV value increased at first diagnosis,ANA+,A-ENA+,immunoglobulin(IgA,IgG,IgM)levels increased,the total number of bone marrow megakarocytes increased,the proportion of bone marrow granules increased,GC+IVIG combined use,and cITP ratio were more common in children aged 4-18 years.The<1 year old group had a higher proportion of onset causes than the other two groups,and the complete remission rate at 1,3,6 and 12 months was higher than the other two groups.
基金项目
中央高水平医院临床科研专项项目(2022-PUMCH-C-040)
内蒙古自治区卫生健康科技计划项目(202201063)