难治性免疫性溶血性贫血，伴有高热幅度、低亲和力IgG抗-Pra冷自身抗体。

Refractory immune hemolytic anemia with a high thermal amplitude, low affinity IgG anti-Pra cold autoantibody.

作者信息

Ramos R R, Curtis B R, Sadler J E, Eby C S, Chaplin H

机构信息

Department of Pathology, Washington University School of Medicine, St. Louis, MO 63110.

出版信息

Autoimmunity. 1992;12(2):149-54. doi: 10.3109/08916939209150322.

DOI:10.3109/08916939209150322

PMID:1617112

Abstract

A 54 y.o. woman presented with acute Coombs-negative hemolytic anemia at an outside hospital where she received 25 RBC transfusions and did not respond to prednisone or splenectomy. On transfer to our hospital, routine DAT and IAT were weakly positive, occasionally negative. When a modified "cold" antiglobulin test was employed, the result was strongly positive for IgG, weakly positive for C3d. Cold agglutinin titer was 32, and the Donath-Landsteiner test was negative. The autoantibody exhibited Pra specificity. The patient failed IV-IgG, high dose IV pulse steroids and cyclophosphamide, and continued to require daily transfusions. She responded 21 days after receiving daily plasma exchange (x3), with pulse cyclophosphamide on the third day, followed by escalating daily oral cyclophosphamide.

摘要

一名54岁女性在外院被诊断为急性库姆斯试验阴性溶血性贫血，在该院接受了25次红细胞输血，对泼尼松或脾切除术均无反应。转至我院后，常规直接抗人球蛋白试验（DAT）和间接抗人球蛋白试验（IAT）呈弱阳性，偶尔为阴性。采用改良“冷”抗球蛋白试验时，结果显示IgG强阳性，C3d弱阳性。冷凝集素效价为32，Donath-Landsteiner试验为阴性。自身抗体表现为Pra特异性。患者对静脉注射免疫球蛋白（IV-IgG）、大剂量静脉脉冲类固醇和环磷酰胺治疗无效，仍需每日输血。在接受每日血浆置换（共3次）21天后出现反应，第三天给予脉冲环磷酰胺，随后逐渐增加口服环磷酰胺剂量。