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[继发于体外可洗脱因子(免疫球蛋白)的阵发性夜间血红蛋白尿]

[Paroxysmal nocturnal hemoglobinuria secondary to an extracorpuscular elutable factor (immunoglobulin)].

作者信息

Audétat F, Teyssier A, Perrin L, Miescher P A

出版信息

Schweiz Med Wochenschr. 1985 Oct 26;115(43):1507-10.

PMID:4081680
Abstract

A case is reported of Coombs-negative hemolytic anemia in the form of transitory PNH associated with acquired immunohemolytic anemia (AIHA). The patient, a 43-year-old man, was admitted to hospital because of jaundice and hemoglobinuria. Investigations revealed hemolytic anemia with the characteristics of PNH (positive Ham test, 50% hemolysis with the sucrose test) and of AIHA (microspherocytosis, survival of isologous red blood cells t1/2 = 9.5 days). The titer of cold agglutinins was not significant. Pre-incubation of the patient's red blood cells with anti-human F (ab')2 caused marked inhibition of hemolysis during the sucrose test. Isologous red blood cells, pre-incubated with an eluate obtained from the patient's erythrocytes, became sensitive to hemolysis during the sucrose test. The sucrose test is pathognomonic for PHN. The "false" positivity in the present case is possibly due to an immunoglobin conferring on the erythrocytes the characteristic property of PNH cells by blocking the activity of the decay accelerating factor.

摘要

报告了一例以短暂性阵发性睡眠性血红蛋白尿(PNH)形式出现的库姆斯试验阴性溶血性贫血病例,该病例与获得性免疫性溶血性贫血(AIHA)相关。患者为一名43岁男性,因黄疸和血红蛋白尿入院。检查发现溶血性贫血具有PNH的特征(酸化血清溶血试验阳性,蔗糖溶血试验50%溶血)和AIHA的特征(小球形红细胞症,同基因红细胞存活时间t1/2 = 9.5天)。冷凝集素滴度不显著。患者红细胞与抗人F(ab')2预孵育后,在蔗糖溶血试验中溶血明显受到抑制。同基因红细胞与从患者红细胞中获得的洗脱液预孵育后,在蔗糖溶血试验中对溶血变得敏感。蔗糖溶血试验对PNH具有诊断意义。本病例中的“假”阳性可能是由于一种免疫球蛋白通过阻断衰变加速因子的活性,赋予红细胞PNH细胞的特征性特性。

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