Schreiber A D, Herskovitz B S, Goldwein M
N Engl J Med. 1977 Jun 30;296(26):1490-4. doi: 10.1056/NEJM197706302962602.
We studied two patients with a low-titer cold-hemagglutinin disease syndrome to investigate the mechanism of hemolysis and the therapeutic response to corticosteroids. The antierythrocyte antibody was of the IgM class, had a high thermal amplitude and had enough activity of 37 degrees C to account for the hemolysis. The capacity of peripheral blood monocytes to increase the osmotic fragility of C3-coated erythrocytes suggests that macrophage interaction with C3-coated erythrocytes explains the observed in vivo spherocytosis. Both patients responded to high-dose corticosteroids. The data suggest that the steroid effect is probably due to alteration of macrophage complement-receptor function. These studies demonstrate the importance of antibody activity at body temperature in producing hemolysis, particularly in this variant of cold-hemagglutinin disease. The response to steroids suggests the efficacy of corticosteroid therapy in alleviating hemolysis due to macrophage recognition of erythrocytes coated with IgM and C3.
我们研究了两名患有低滴度冷凝集素病综合征的患者，以探究溶血机制及对皮质类固醇的治疗反应。抗红细胞抗体为IgM类，具有高热幅度，且在37℃时有足够活性来解释溶血现象。外周血单核细胞增加C3包被红细胞渗透脆性的能力表明，巨噬细胞与C3包被红细胞的相互作用解释了所观察到的体内球形红细胞增多现象。两名患者对大剂量皮质类固醇均有反应。数据表明，类固醇效应可能是由于巨噬细胞补体受体功能改变所致。这些研究证明了体温下抗体活性在产生溶血中的重要性，尤其是在这种冷凝集素病变体中。对类固醇的反应表明皮质类固醇疗法在减轻因巨噬细胞识别IgM和C3包被红细胞而导致的溶血方面具有疗效。
