与针对血型糖蛋白A上或与之相关的决定簇的IgM温自身抗体相关的严重自身免疫性溶血性贫血。
Severe autoimmune hemolytic anemia associated with IgM warm autoantibodies directed against determinants on or associated with glycophorin A.
作者信息
Garratty G, Arndt P, Domen R, Clarke A, Sutphen-Shaw D, Clear J, Groncy P
机构信息
American Red Cross Blood Services, Southern California Region, Los Angeles 90006, USA.
出版信息
Vox Sang. 1997;72(2):124-30. doi: 10.1046/j.1423-0410.1997.7220124.x.
BACKGROUND AND OBJECTIVES
Autoimmune hemolytic anemiA (AIHA) is rarely caused by IgM warm autoantibodies, and is sometimes difficult to diagnose. We describe three patients with severe AIHA caused by IgM warm autoantibodies; in two of the three, the hemolysis was fatal.
MATERIALS AND METHODS
Conventional hematologic and serologic procedures were used.
RESULTS
The IgM autoantibodies in all three cases were directed against determinants on or associated with glycophorin A (Ena, Wrb, and Pr). The IgM autoantibodies and unusual serological characteristics, in that the agglutinins were detected or greatly enhanced only by the presence of albumin or a low pH, and/or the agglutinins reacted optimally at 20-30 degrees C.
CONCLUSIONS
IgM warm autoantibodies directed at determinants on or associated with glycophorin A appear to cause severe (often fatal) hemolytic anemia.
背景与目的
自身免疫性溶血性贫血（AIHA）很少由IgM温抗体引起，有时难以诊断。我们描述了3例由IgM温抗体引起的严重AIHA患者；其中3例中的2例溶血是致命的。
材料与方法
采用传统血液学和血清学方法。
结果
所有3例中的IgM自身抗体均针对血型糖蛋白A上或与之相关的决定簇（Ena、Wrb和Pr）。IgM自身抗体具有不寻常的血清学特征，即仅在白蛋白存在或低pH条件下凝集素才被检测到或显著增强，和/或凝集素在20-30℃时反应最佳。
结论
针对血型糖蛋白A上或与之相关决定簇的IgM温抗体似乎会引起严重（通常是致命的）溶血性贫血。
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