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红细胞自身抗体、自身免疫性溶血和骨髓增生异常综合征。

Erythrocyte autoantibodies, autoimmune haemolysis, and myelodysplastic syndromes.

作者信息

Sokol R J, Hewitt S, Booker D J

机构信息

Regional Blood Transfusion Centre, Sheffield.

出版信息

J Clin Pathol. 1989 Oct;42(10):1088-91. doi: 10.1136/jcp.42.10.1088.

Abstract

Immunohaematological investigations were carried out in 46 patients with erythrocyte autoantibodies associated with myelodysplastic syndromes. Eight patients had refractory anaemia, 17 refractory anaemia with ring sideroblasts, 11 refractory anaemia with excess of blasts, four chronic myelomonocytic leukaemia, five refractory anaemia with excess of blasts in transformation and one could not be classified. Standard agglutination direct antiglobulin tests showed that the red cells were most often coated with IgG and C3d, though increased amounts of IgM or IgA were also found in 15 of 35 cases (43%) when the more sensitive enzyme linked method was used. The IgG antibodies were predominantly of IgG1 subclass. Clinically important autoimmune haemolysis occurred in 15 patients, and was of "warm", "cold," and "mixed" types in seven, four, and four cases, respectively: it is important to recognise its presence in view of the good response to treatment. The increased incidence of erythrocyte autoantibodies in myelodysplastic syndromes is thought to be one manifestation of disturbed immune homeostasis.

摘要

对46例伴有骨髓增生异常综合征的红细胞自身抗体患者进行了免疫血液学检查。8例为难治性贫血,17例为环形铁粒幼细胞性难治性贫血,11例为原始细胞过多的难治性贫血,4例为慢性粒单核细胞白血病,5例为转化型原始细胞过多的难治性贫血,1例无法分类。标准凝集直接抗球蛋白试验显示,红细胞最常被IgG和C3d包被,不过当使用更敏感的酶联法时,在35例中的15例(43%)也发现了IgM或IgA量的增加。IgG抗体主要为IgG1亚类。15例患者发生了具有临床意义的自身免疫性溶血,分别有7例、4例和4例为“温抗体型”、“冷抗体型”和“混合型”:鉴于对治疗反应良好,认识到其存在很重要。骨髓增生异常综合征中红细胞自身抗体发生率的增加被认为是免疫稳态紊乱的一种表现。

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