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儿童期与非补体结合IgM自身抗体相关的自身免疫性溶血性贫血。

Autoimmune haemolytic anaemia in childhood associated with non-complement binding IgM autoantibodies.

作者信息

Salama A, Mueller-Eckhardt C

出版信息

Br J Haematol. 1987 Jan;65(1):67-71. doi: 10.1111/j.1365-2141.1987.tb06137.x.

Abstract

Red cell bound IgM autoantibodies free of associated IgG autoantibodies and unable to activate complement have not yet been considered as the cause of autoimmune haemolytic anaemia (AIHA). We report on warm type AIHA resulting from the action of IgM autoantibodies on circulating RBC. Twelve children (eight of whom were infants) with relatively severe haemolytic anaemia were studied. Whereas the clinical findings and courses of all children appeared to be compatible with warm type AIHA, the serological findings during the haemolytic phase were atypical in that the direct antiglobulin test (DAT) was negative in 11, and positive in one case due to C3d only. The use of radiolabelled antihuman globulin antibodies showed, however, that the RBC of all the patients were sensitized with warm IgM antibodies. Elevated values of IgA and/or IgG immunoglobulins on the patients' RBC were found to be present in only two cases. Complement activation by IgM autoantibodies could not be detected in all other cases neither in vivo nor in vitro. Thus, we conclude that non-complement binding IgM autoantibodies were responsible for the AIHA in the majority of these children possibly representing an as yet unrecognized variant of warm type AIHA.

摘要

不伴有相关IgG自身抗体且无法激活补体的红细胞结合IgM自身抗体尚未被视为自身免疫性溶血性贫血(AIHA)的病因。我们报告了由IgM自身抗体作用于循环红细胞导致的温抗体型AIHA。对12名患有相对严重溶血性贫血的儿童(其中8名是婴儿)进行了研究。尽管所有儿童的临床表现和病程似乎都与温抗体型AIHA相符,但溶血期的血清学检查结果并不典型,因为11例直接抗球蛋白试验(DAT)为阴性,仅1例因C3d呈阳性。然而,使用放射性标记的抗人球蛋白抗体显示,所有患者的红细胞均被温IgM抗体致敏。仅在2例患者的红细胞上发现IgA和/或IgG免疫球蛋白值升高。在所有其他病例中,无论是体内还是体外,均未检测到IgM自身抗体激活补体。因此,我们得出结论,非补体结合IgM自身抗体是这些儿童中大多数AIHA的病因,可能代表一种尚未被认识的温抗体型AIHA变体。

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