Moake J L, Schultz D R
Am J Med. 1975 Mar;58(3):431-7. doi: 10.1016/0002-9343(75)90610-5.
A 37 year old woman with extravascular hemolytic anemia had a positive Monospot test associated with positive antiglobulin and anticomplement Coombs' tests, cold agglutinins and warm autoantibodies. IgG-kappa (k) antibodies, which reacted with all panel red cells at 37 degrees C, were eluted from her circulating red cells. However, neither immunoglobulins nor C3 was detected after her serum was adsorbed with heterologous red cell stroma at 37 degrees C and eluted at the same temperature in glycine buffer. In contrast, IgM-kappa and IgM-lambda (lambda), IgG-3-kappa, IgG4-lambda, IgA-lambda and C3 were eluted at 37 degrees C from heterologous red cell stroma after adsorption with her serum at 0 degrees C. Thus, antibodies of several types, which were present in the patient's serum, reacted optimally with red cell antigens at low temperature. Cold-reactive IgG3-kappa antibodies, which also capable of interacting with red cells at 37 degrees C, probably accounted for the IgG-kappa antibodies eluted from the patient's circulating red cells. The patient's serum C4 titers were decreased, with low normal to moderately depressed C3 and low normal C5, indicating that the anti-red cell IgM and/or IgG3-kappa antibodies probably fixed complement. A localized cold stress test resulted in a transient increase in plasma hemoglobin and a decrease in serum C3 titer. These findings, and the beneficial clinical response obtained with small doses of prednisone, suggest that both the cold-reactive antibodies and the IgG-kappa on circulating red cells were pathophysiologically significant. This is the first report of a patient with multiple red cell autoantibodies in whom serum complement component titers were determined in conjunction with characterization of the anti-red cell immunoglobulins. Subclinical infectious mononucleosis may have preceded the prolonged hemolytic episode. Clinical evidence of systemic lupus erythematosus has not appeared.
一名患有血管外溶血性贫血的37岁女性，其嗜异性凝集试验呈阳性，抗球蛋白和抗补体库姆斯试验、冷凝集素和温自身抗体均为阳性。从她循环的红细胞中洗脱出血清中与所有谱细胞在37℃反应的IgG-κ（k）抗体。然而，当她的血清在37℃用异源红细胞基质吸附并在相同温度的甘氨酸缓冲液中洗脱后，未检测到免疫球蛋白和C3。相比之下，在0℃用她的血清吸附后，IgM-κ和IgM-λ（λ）、IgG-3-κ、IgG4-λ、IgA-λ和C3在37℃从异源红细胞基质中洗脱。因此，患者血清中存在的几种类型的抗体在低温下与红细胞抗原反应最佳。也能够在37℃与红细胞相互作用的冷反应性IgG3-κ抗体可能是从患者循环红细胞中洗脱的IgG-κ抗体的原因。患者的血清C4滴度降低，C3低至正常至中度降低，C5正常偏低，表明抗红细胞IgM和/或IgG3-κ抗体可能固定补体。局部冷应激试验导致血浆血红蛋白短暂升高和血清C3滴度降低。这些发现以及小剂量泼尼松获得的有益临床反应表明，冷反应性抗体和循环红细胞上的IgG-κ在病理生理上具有重要意义。这是第一例报告了多种红细胞自身抗体患者，同时测定了血清补体成分滴度并对抗红细胞免疫球蛋白进行了表征。亚临床传染性单核细胞增多症可能先于长期溶血发作。尚未出现系统性红斑狼疮的临床证据。
