【摘要】：目的:研究兒童自身免疫性溶血性貧血(autoimmune hemolytic anemia,AIHA)的臨床特征、實驗室特點及治療效果,加深對該疾病的認(rèn)識,為臨床診斷、治療提供參考。方法:共研究68例AIHA患兒(包括Evans綜合征20例),所有病例均接受糖皮質(zhì)激素治療,部分患兒接受靜脈注射免疫球蛋白(intravenous immunoglobulin,IVIG)及免疫抑制劑等治療,對臨床表現(xiàn)、實驗室檢查結(jié)果及治療轉(zhuǎn)歸進(jìn)行回顧分析。結(jié)果:(1)原發(fā)性AIHA 27例,占39.7%,繼發(fā)性AIHA 41例,占60.3%;Evans綜合征(ES)20例,占29.4%;小于3歲患兒占63.2%。(2)發(fā)病時溶血程度比較:發(fā)病年齡≤1歲組血紅蛋白水平低于1歲組,復(fù)合抗體組低于單一抗體組,含IgM抗體組低于不含Ig M抗體組(P均0.05);比較游離血紅蛋白水平:單純AIHA組與ES組,≤1歲組與1歲組,復(fù)合抗體組與單一抗體組,含IgM抗體組與不含Ig M抗體組之間無統(tǒng)計學(xué)差異(P均0.05);間接膽紅素水平比較:單純AIHA組高于ES組(P0.05)。(3)治療時間比較:ES組長于單純AIHA組(P0.05),≤1歲組與1歲組、單一抗體組與復(fù)合抗體組、含IgM組與不含IgM組、應(yīng)用IVIG組與未用IVIG組之間治療時間無統(tǒng)計學(xué)差異(P0.05)。(4)45例回訪資料完整的患兒中,首次發(fā)病后應(yīng)用糖皮質(zhì)激素治療均達(dá)到緩解,其中29例(64.4%)持續(xù)緩解中,16例(35.6%)在首次緩解后復(fù)發(fā),復(fù)發(fā)率31.5%,9例(56.3%)復(fù)發(fā)后糖皮質(zhì)激素治療仍然有效。比較單純AIHA組與ES組、≤1歲組與1歲組、單一抗體組與復(fù)合抗體組、含IgM抗體組與不含IgM抗體組、應(yīng)用IVIG組與未用IVIG組復(fù)發(fā)率,均無統(tǒng)計學(xué)差異(P0.05)。結(jié)論:發(fā)病年齡、不同抗體類型及是否為ES可為臨床判斷病情嚴(yán)重程度及治療效果提供依據(jù)。對于初次發(fā)病及復(fù)發(fā)患兒,糖皮質(zhì)激素治療兒童AIHA療效確切;ES患兒需要更長的治療時間;靜脈注射免疫球蛋白未能改善治療效果。
[Abstract]:Objective: to study the clinical features, laboratory characteristics and therapeutic effects of autoimmune hemolytic anemia (autoimmune hemolytic anemia,AIHA) in children, and to provide reference for clinical diagnosis and treatment. Methods: 68 cases of AIHA (including 20 cases of Evans syndrome) were studied. All of them were treated with glucocorticoid, and some of them received intravenous immunoglobulin (intravenous immunoglobulin,IVIG) and immunosuppressant. The results of laboratory examination and the outcome of treatment were analyzed retrospectively. Results: (1) there were 27 cases of primary AIHA (39.7%), 41 cases of secondary AIHA (60.33%), 20 cases of (ES) (29.4%), and 63.2% of children younger than 3 years old. (2) the hemolysis degree of the age 鈮，

本文編號：2212656