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与温反应性IgM自身抗体相关的致命性免疫性溶血性贫血和肝衰竭。

Fatal immune hemolytic anemia and hepatic failure associated with a warm-reacting IgM autoantibody.

作者信息

Shirey R S, Kickler T S, Bell W, Little B, Smith B, Ness P M

出版信息

Vox Sang. 1987;52(3):219-22. doi: 10.1111/j.1423-0410.1987.tb03031.x.

Abstract

Autoimmune hemolytic anemia (AIHA) caused by warm-reacting IgM autoantibodies is rare. We report a fatal case of primary AIHA with a warm-reacting IgM autoantibody. Recurrent episodes of intravascular hemolysis, unresponsive to all therapy and progressive hepatic dysfunction characterized the patient's clinical course. Despite corticosteroid therapy, splenectomy and multiple blood transfusions, the patient died from liver failure. The IgM autoantibody caused autoagglutination of the patient's red cells at 37 degrees C. Eluates prepared from the patient's red cells agglutinated saline-suspended test cells without the addition of antiglobulin reagent. We propose that warm-reacting IgM antibodies may lead to in vivo autoagglutination and may be associated with hepatic failure.

摘要

由温反应性IgM自身抗体引起的自身免疫性溶血性贫血(AIHA)较为罕见。我们报告了一例原发性AIHA伴温反应性IgM自身抗体的致死病例。血管内溶血反复发作,对所有治疗均无反应,且肝功能进行性损害是该患者的临床病程特点。尽管接受了皮质类固醇治疗、脾切除术和多次输血,患者仍死于肝功能衰竭。该IgM自身抗体在37℃时导致患者红细胞自身凝集。从患者红细胞制备的洗脱液在不添加抗球蛋白试剂的情况下使盐水悬浮的试验细胞发生凝集。我们提出,温反应性IgM抗体可能导致体内自身凝集,并可能与肝功能衰竭有关。

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