Petz L D, Yam P, Wilkinson L, Garratty G, Lubin B, Mentzer W
Blood. 1984 Jul;64(1):301-4.
We have used the complement-fixing antibody consumption ( CFAC ) test to detect small concentrations of IgG on red blood cells from patients with hemolytic anemias that are not thought to be caused by an immune mechanism. Although patients with hereditary spherocytosis, pyruvate kinase deficiency, and mechanical hemolytic anemias generally had normal concentrations of IgG bound to their red cells (less than 25 molecules IgG per red cell), we found that 39/62 (63%) patients with sickle cell anemia had elevated values. These 39 patients had a mean of 195 and a maximum of 890 molecules of IgG per red cell. None of the patients had been transfused within the previous 90 days, and some had never been transfused. Direct antiglobulin tests were positive in only two instances and autoantibodies were not found in the serum of any patient. However, eluates from the red cells of 6 of 23 patients demonstrated antibody activity against all of a panel of normal red cells by the indirect antiglobulin test. There was no correlation between the number of IgG molecules on patients' red cells and the severity of their anemia, the incidence of painful sickle cell crises, the reticulocyte count, or with blood transfusion history. We conclude that further study of immunohematologic abnormalities in patients with sickle cell anemia is warranted, especially in view of previous reports in this population of patients with red cell autoantibodies, autoimmune hemolytic anemia, hemolytic transfusion reactions without detectable alloantibodies, and an association of some episodes of pain crises with immunologically mediated red cell destruction.
我们运用补体结合抗体消耗(CFAC)试验,来检测溶血性贫血患者红细胞上低浓度的IgG,这些溶血性贫血被认为并非由免疫机制所致。尽管遗传性球形红细胞增多症、丙酮酸激酶缺乏症以及机械性溶血性贫血患者,其红细胞结合的IgG浓度通常正常(每个红细胞结合的IgG少于25个分子),但我们发现,62例镰状细胞贫血患者中有39例(63%)的该数值升高。这39例患者每个红细胞平均结合195个IgG分子,最多达890个。所有患者在过去90天内均未接受输血,部分患者从未输过血。直接抗球蛋白试验仅在两例中呈阳性,且所有患者血清中均未发现自身抗体。然而,23例患者中有6例红细胞洗脱液通过间接抗球蛋白试验显示对一组正常红细胞具有抗体活性。患者红细胞上IgG分子数量与贫血严重程度、镰状细胞疼痛危象发生率、网织红细胞计数或输血史之间均无相关性。我们得出结论,鉴于此前关于该人群中红细胞自身抗体、自身免疫性溶血性贫血、无可检测到的同种抗体的溶血性输血反应以及某些疼痛危象发作与免疫介导的红细胞破坏之间存在关联的报道,有必要对镰状细胞贫血患者的免疫血液学异常进行进一步研究。