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自身免疫性溶血:温抗体与冷抗体混合型。

Autoimmune hemolysis: mixed warm and cold antibody type.

作者信息

Sokol R J, Hewitt S, Stamps B K

出版信息

Acta Haematol. 1983;69(4):266-74. doi: 10.1159/000206903.

Abstract

25 patients with mixed autoimmune haemolysis are described; in these cases the combined criteria for warm antibody autoimmune haemolysis and also the cold agglutinin syndrome were present. The direct antiglobulin tests were positive with IgG and complement coating of the red cells. The IgM and IgG components could be separated. The cold autoantibodies were active at 30 degrees C or above and often showed blood group specificity within the Ii system; specificity was not usually evident in the warm component. Haemolysins were found in 18 patients. Serologically, the condition maintained its mixed nature throughout; only 2 cases later changed to a warm type picture. Mixed autoimmune haemolysis was found in all age groups but was more frequent in later life; the male:female ratio was 1:15. The cases were either idiopathic (44%) or secondary; in the latter, systemic lupus erythematosus or lymphoma were the most frequently associated disorders. The patients tended to have severe haemolysis which, although usually responding well to treatment, ran a chronic course with intermittent exacerbations, thus making overall management difficult; plasma exchange may be a useful adjunct to chemotherapy.

摘要

本文描述了25例混合性自身免疫性溶血患者;这些病例同时具备温抗体自身免疫性溶血和冷凝集素综合征的综合标准。直接抗球蛋白试验显示红细胞上有IgG和补体包被,呈阳性。IgM和IgG成分可以分离。冷自身抗体在30℃或更高温度下具有活性,并且在Ii系统内通常表现出血型特异性;温成分中通常没有明显的特异性。18例患者发现有溶血素。血清学上,这种情况始终保持其混合性质;只有2例后来转变为温型表现。混合性自身免疫性溶血在各年龄组均有发现,但在老年时更为常见;男女比例为1:15。这些病例要么是特发性的(44%),要么是继发性的;在继发性病例中,系统性红斑狼疮或淋巴瘤是最常伴发的疾病。患者往往有严重的溶血,尽管通常对治疗反应良好,但病程呈慢性,伴有间歇性加重,因此整体管理困难;血浆置换可能是化疗的有用辅助手段。

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