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对自身免疫性溶血性贫血(AIHA)36例进行分析,结果多数起病急骤(24例);发病诱因为呼吸道感染者多(18例);临床表现主要为苍白、黄疸、尿深黄、肝脾肿大;外周血象示血红蛋白降低,网织红细胞升高;骨髓象示有核细胞增生明显活跃31例,红系以中、晚幼红居多,可见巨幼样变及分裂象,粒红比例倒置。胆红素增高32例,以间接胆红素为主;Coomb试验阳性率为56.23％,其中以温型抗体者多见,激素治疗见效快。温冷双抗体者治疗效果差,病程迁延,易复发。Coomb试验是否阳性与临床病情、预后、年龄无关。本组病例曾动态观察1例患儿Coomb试验,入院时滴度为2068,6个月时为512,18个月时为256,24个月时为128,但无任何临床表现,血红蛋白及网织红细胞均在正常范围,提示AI-HA易复发原因可能与抗体消失慢有关。本组有4例患儿激素治疗不满2周,Hb<90g/L,网织红细胞>0.05,提示近期效果与激素治疗时间长短有关。输洗涤红细胞者贫血纠正快,输全血者效果慢,单用激素治疗无效者可加用丙种球蛋白,能取得满意的效果,防治感染是预防复发的关键。
Thirty-six cases of autoimmune hemolytic anemia (AIHA) were analyzed. The majority of cases were found to be abrupt (24 cases); the predisposing causes were mostly respiratory tract infections (18 cases); the clinical manifestations were pale, jaundice, Splenomegaly; peripheral blood showed hemoglobin decreased, reticulocyte increased; bone marrow showed nuclear hyperplasia was significantly active in 31 cases, the red line to the late, mostly late juvenile, visible megaloblastic degeneration and mitotic figure, the proportion of red Inverted. Elevated bilirubin in 32 cases, mainly indirect bilirubin; Coomb test positive rate was 56.23%, of which warm-type antibodies are more common, hormone treatment quicker. Cold double antibody treatment is poor, duration of the disease, easy to relapse. Coomb test is positive and clinical condition, prognosis, age has nothing to do. This group of patients had a dynamic observation of Coomb test in 1 child, admission titers of 2068, 6 months, 512, 18 months, 256, 24 months, when 128, but without any clinical manifestations, hemoglobin and the net Reticulocytes are in the normal range, suggesting that AI-HA easy to relapse may be related to the slow disappearance of antibodies. This group had 4 cases of children less than 2 weeks of hormone therapy, Hb <90g / L, reticulocyte> 0.05, suggesting that the recent effect of hormone therapy and the length of time. Red blood cells lose red blood cells to correct anemia, the effect of slow blood transfusions, hormone therapy alone can be added with gamma globulin, can achieve satisfactory results, prevention and treatment of infection is the key to preventing recurrence.