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病例报告:IgG自身抗N抗体作为严重自身免疫性溶血性贫血的病因

A case report: IgG autoanti-N as a cause of severe autoimmune hemolytic anemia.

作者信息

Combs M R, Telen M J, Hall S E, Rosse W F

机构信息

Immunohematology Laboratory, Duke University Medical Center, Box 2928, Durham, NC 27710, USA.

出版信息

Immunohematology. 1990;6(4):83-6.

Abstract

A 21-year-old white worn was referred for evaluation of hemolytic anemia after a 9-day history of marked hemoglobinuria, jaundice, and weakness. The patient's hematocrit was 18%, despite at least eight transfusions over the previous week, and the reticulocyte count was < 1%. Serologic evaluation revealed a weakly positive direct antiglobulin test with anti-C3 only The serum contained cold and warm-reacting anti-N Dithiothreitol had no effect on either the cold- or the warm-reacting anti-N activity, and radioimmunoassay with monoclonal anti-IgG was strongly positive, indicating that both the cold- and the warm-reacting anti-N reactivity resided in the IgG fraction. The patient was treated with N - 'N' + red cell transfusions, prednisone, and azathioprine and gradually became transfusion independent. Postrecovery typing revealed her red cells to be M+N+S+s+. This constitutes the third case of autoimmune hemolytic anemia associated with IgG autoanti-N. The marked hemoglobinuria and reticulocytopenia are unique features of this case.

摘要

一名21岁白人女性因明显血红蛋白尿、黄疸和虚弱9天而被转诊进行溶血性贫血评估。尽管在过去一周内至少输血8次,患者的血细胞比容仍为18%,网织红细胞计数<1%。血清学评估显示直接抗球蛋白试验弱阳性,仅抗C3阳性。血清中含有冷反应和温反应抗N。二硫苏糖醇对冷反应或温反应抗N活性均无影响,单克隆抗IgG放射免疫测定呈强阳性,表明冷反应和温反应抗N反应性均存在于IgG组分中。该患者接受了N - 'N' +红细胞输血、泼尼松和硫唑嘌呤治疗,并逐渐不再依赖输血。恢复后血型鉴定显示她的红细胞为M+N+S+s+。这是第三例与IgG自身抗N相关的自身免疫性溶血性贫血病例。明显的血红蛋白尿和网织红细胞减少是该病例的独特特征。

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