应用唐奈斯-兰施泰纳试验诊断阵发性寒冷性血红蛋白尿症。
Applying Donath-Landsteiner test for the diagnosis of paroxysmal cold hemoglobinuria.
作者信息
Tiwari Aseem Kumar, Aggarwal Geet, Mitra Subhasis, Arora Dinesh, Bhardwaj Gunjan, Ratan Ankita, Setya Divya
机构信息
Department of Transfusion Medicine, Medanta-The Medicity, Gurgaon, Haryana, India.
出版信息
Asian J Transfus Sci. 2020 Jan-Jun;14(1):57-59. doi: 10.4103/ajts.AJTS_132_17. Epub 2020 Jul 24.
61-year old male patient was admitted to the hospital with clinical picture of hemolytic anemia with hemoglobinuria. Patient was suspected to have Infectious Mononucleosis (IM) with Auto Immune Hemolytic Anemia (AIHA). DAT was positive with anti-C3d specificity. Donath Landsteiner (DL) test was positive; confirming Paroxysmal Cold Hemoglobinuria (PCH). The final diagnosis was IM with PCH. Patient was managed conservatively and discharged after seven days. DL test specifically detects a biphasic autoantibody (IgG type) that binds to RBCs at cold temperatures, and fixes complement on the RBC membrane. However RBCs are only lysed upon warming to 37C when complement cascade proceeds to completion.
一名61岁男性患者因溶血性贫血伴血红蛋白尿的临床表现入院。患者疑似患有传染性单核细胞增多症（IM）合并自身免疫性溶血性贫血（AIHA）。直接抗人球蛋白试验（DAT）以抗C3d特异性呈阳性。Donath Landsteiner（DL）试验呈阳性；确诊为阵发性冷血红蛋白尿（PCH）。最终诊断为IM合并PCH。患者接受保守治疗，七天后出院。DL试验专门检测一种双相自身抗体（IgG型），该抗体在低温下与红细胞结合，并在红细胞膜上固定补体。然而，只有在升温至37℃时补体级联反应完成，红细胞才会被溶解。
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