Kannagi R, Mitsuoka C
Laboratory of Experimental Pathology, Aichi Cancer Center.
Nihon Rinsho. 1996 Sep;54(9):2519-27.
Recent progress on the studies of cold agglutinin disease and paroxysmal cold hemoglobinuria is reviewed. In both types of autoimmune hemolytic anemia, auto-antibodies are directed specifically to the antigenic epitopes which have physiological significance as differentiation antigens. They are the metabolic precursors for the synthesis of strong allo-antigens such as ABO(H). Although both antigen epitopes, i.e., differentiation and allo-antigen epitopes, are present on the red blood cells, the autoimmune reaction is directed specifically to the differentiation antigen epitopes, and not directed to the strong allo-antigenic epitopes. Most of auto-antigens also serve as receptors for some viruses, bacteria and drugs. Recent analysis on the sequence of auto-antibodies indicated that most of the auto-antibodies in cold agglutinin disease are encoded by the VH-4-21 gene of VH4 family, indicating the auto-antibodies are produced by dysregulation of very limited B cell clones. This implies that immune system is prone to be disturbed with self differentiation antigens rather than strong self allo-antigens, and that the dysfunction of auto-reactive B cell clones could be triggered by infection of some viruses and bacteria, or by certain medication.
本文综述了冷凝集素病和阵发性冷性血红蛋白尿的研究进展。在这两种自身免疫性溶血性贫血中，自身抗体特异性地针对具有作为分化抗原的生理意义的抗原表位。它们是合成如ABO(H)等强同种抗原的代谢前体。尽管这两种抗原表位，即分化抗原表位和同种抗原表位，都存在于红细胞上，但自身免疫反应特异性地针对分化抗原表位，而不针对强同种抗原表位。大多数自身抗原也是某些病毒、细菌和药物的受体。最近对自身抗体序列的分析表明，冷凝集素病中的大多数自身抗体由VH4家族的VH-4-21基因编码，这表明自身抗体是由非常有限的B细胞克隆失调产生的。这意味着免疫系统更容易被自身分化抗原而非自身强同种抗原干扰，并且自身反应性B细胞克隆的功能障碍可能由某些病毒和细菌感染或特定药物触发。
