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在日本,自身免疫性溶血性贫血(AIHA)是一种较少见的疾病。1975年的流行病学调查结果表明:所有溶血性贫血估计总发病率为10.3～36.6/100万日本人。其中AIHA约占1/3(2.7～9.7/100万人)。90%的AIHA是温热型,而冷型AIHA(冷凝素病和阵发牲寒冷性血红蛋白尿)罕见。特发性与继发性AIHA的比例约为5:1。10%的AIHA伴有血小板减少,称为Evans综合征。年龄分布在20岁左右呈高峰,男/女比例为1.0/2.86。然而,最近的资料表明,在老年期也有一高峰,此种病例男/女比值接近1:0。有关AIHA的发病机理,即何以产生自身抗体仍未明了。曾提出过几种假说:如:1.选择性抑制性T细胞;2.多克隆B细胞激活;3.B细胞的单克隆增
In Japan, autoimmune hemolytic anemia (AIHA) is a less common disease. Epidemiological findings from 1975 showed that the overall incidence of all hemolytic anemias was between 10.3 and 36.6 per 100 000 Japanese. AIHA accounted for about 1/3 (2.7 ~ 9.7 / 100 million). 90% of AIHA is warm-type, while cold-type AIHA (cholestatic disease and paroxysmal cold hemoglobinuria) is rare. Idiopathic and secondary AIHA ratio of about 5: 1.10% of AIHA associated with thrombocytopenia, known as Evans syndrome. The age distribution peaked at about age 20 with a male / female ratio of 1.0 / 2.86. However, recent data indicate that there is also a peak in old age, in which case the male / female ratio approaches 1: 0. The pathogenesis of AIHA, how to produce autoantibodies remains unclear. Several hypotheses have been proposed such as: 1. Selective inhibitory T cells; 2. Polyclonal B cell activation; 3. Monoclonal augmentation of B cells