Winiarski J
Department of Paediatrics, Karolinska Institute, Huddinge Hospital, Stockholm, Sweden.
Br J Haematol. 1989 Sep;73(1):88-92. doi: 10.1111/j.1365-2141.1989.tb00225.x.
In contrast to the situation in chronic idiopathic thrombocytopenic purpura (ITP) it has not been known whether acute childhood ITP has an autoimmune aetiology and to what extent autoantibodies directed to platelet autoantigens appear during the transient course of this disease. In the present study of 21 children with acute ITP an immunoblot technique was applied to detect serum antibodies to electrophoretically (SDS-PAGE) separated normal donor platelet membrane proteins. Platelet antigen binding antibodies detected by alkaline phosphatase conjugated protein A or anti-IgM antibody were observed in 13 out of 21 (62%) patients while controls were negative. In nine children antibodies were directed to antigens localized on the three major membrane glycoproteins. GPIb (four), GPIIb (one) and GPIIIa (five). Antibodies to a 250 kDa antigen were noted in one case and to smaller 25-52 kDa proteins in 12 patients. Four patients had IgG as well as IgM platelet antibodies while in nine only IgM was found. The reactions were eliminated after absorptions of sera with fresh platelets. In all of three tested patients the glycoprotein antigen specific antibodies could be detected in acid eluates prepared from the absorbing platelets. The presence of antibodies, predominantly IgM, to platelet surface antigens in a majority of the children with acute ITP, may suggest an autoimmune process, albeit transitory, similar to that in chronic ITP.
与慢性特发性血小板减少性紫癜(ITP)的情况不同,儿童急性ITP是否具有自身免疫病因以及在这种疾病的短暂病程中针对血小板自身抗原的自身抗体出现的程度尚不清楚。在本项针对21例急性ITP患儿的研究中,应用免疫印迹技术检测血清中针对经电泳(SDS-PAGE)分离的正常供体血小板膜蛋白的抗体。通过碱性磷酸酶偶联的蛋白A或抗IgM抗体检测到血小板抗原结合抗体的患者有13例(62%),而对照组均为阴性。在9名儿童中,抗体针对位于三种主要膜糖蛋白上的抗原,即糖蛋白Ib(GPIb,4例)、糖蛋白IIb(GPIIb,1例)和糖蛋白IIIa(GPIIIa,5例)。1例患者检测到针对250 kDa抗原的抗体,12例患者检测到针对25 - 52 kDa较小蛋白的抗体。4例患者同时存在IgG和IgM血小板抗体,9例患者仅发现IgM。用新鲜血小板吸收血清后反应消失。在所有3例受试患者中,从吸收血小板制备的酸洗脱液中均可检测到糖蛋白抗原特异性抗体。大多数急性ITP患儿存在针对血小板表面抗原的抗体,主要是IgM,这可能提示存在一种类似于慢性ITP的自身免疫过程,尽管是短暂的。