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导致同种免疫性新生儿中性粒细胞减少症的粒细胞抗体的血清学和临床特征

Serological and clinical aspects of granulocyte antibodies leading to alloimmune neonatal neutropenia.

作者信息

Bux J, Jung K D, Kauth T, Mueller-Eckhardt C

机构信息

Institute for Clinical Immunology and Transfusion Medicine, Justus-Liebig-University, Giessen, Germany.

出版信息

Transfus Med. 1992 Jun;2(2):143-9. doi: 10.1111/j.1365-3148.1992.tb00148.x.

Abstract

Eighteen cases of alloimmune neonatal neutropenia (ANN) were analysed for their clinical and serological properties. Pregnancy was normal in all cases, but a 50% incidence of abortion is recorded. With the exception of two premature babies, all newborns were delivered at term. Omphalitis and mild infections of the skin were predominantly present. None of the new-borns died by overwhelming sepsis. The average duration of neutropenia was 11 weeks (range 3-28 weeks). Intravenous IgG therapy was followed by transient remission in 2 of 4 affected newborns. Antibody differentiation revealed in five sera NA1-, in four sera NA2- and in two sera NB1-specific antibodies. In two sera only HLA antibodies were detectable. Complement activating antibodies were determined in 72% of the sera. Screening for granulocyte-specific antibodies in 1016 postpartum sera of unselected women revealed a total of 11 sera (1.1%) reacting selectively with granulocytes, but only four (0.4%) were directed against a known granulocyte-specific antigen. None of the new-born of mothers alloimmunized to granulocyte antigens developed neutropenia, which suggests an incidence of ANN below 0.1%.

摘要

对18例同种免疫性新生儿中性粒细胞减少症(ANN)病例的临床和血清学特性进行了分析。所有病例妊娠均正常,但有50%的流产发生率记录。除2例早产儿外,所有新生儿均足月分娩。主要表现为脐炎和轻度皮肤感染。没有新生儿死于严重败血症。中性粒细胞减少的平均持续时间为11周(范围3 - 28周)。4例受影响的新生儿中有2例在静脉注射免疫球蛋白治疗后出现短暂缓解。抗体鉴别显示,5份血清中有NA1特异性抗体,4份血清中有NA2特异性抗体,2份血清中有NB1特异性抗体。在2份血清中仅可检测到HLA抗体。72%的血清中检测到补体激活抗体。在1016份未选择的产后妇女血清中筛查粒细胞特异性抗体,共发现11份血清(1.1%)与粒细胞选择性反应,但只有4份(0.4%)针对已知的粒细胞特异性抗原。粒细胞抗原同种免疫的母亲所生的新生儿均未发生中性粒细胞减少症,这表明ANN的发生率低于0.1%。

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