Philippe Pierre
Service de médecine interne, Hôtel-Dieu, CHU de Clermont-Ferrand, Clermont-Ferrand, France.
Presse Med. 2007 Dec;36(12 Pt 3):1959-69. doi: 10.1016/j.lpm.2007.03.035. Epub 2007 May 3.
Autoimmune hemolytic anemia (AIHA) is diagnosed in the presence of anemia, usually macrocytic and of variable intensity, reticulocytosis, and a positive direct and/or indirect antiglobulin test, after ruling out other types of hemolytic anemia. A positive direct antiglobulin test alone is not sufficient to diagnose AIHA and may be positive in many patients without anemia or negative in some patients with AIHA. AIHA may be classified into two major categories according to the optimal temperature of antibody activity: warm-reacting autoantibodies (usually IgG) optimal around 37 degrees C and cold-reacting autoantibodies, optimal at 4 degrees C (usually IgM). This classification guides the selection of tests and treatment. AIHA is widely reported to be associated with a variety of other diseases, although these associations are often fortuitous. A minimal set of useful investigations is appropriate since AIHA may be secondary to viral infections, lymphoid malignancies, or autoimmune disorders such as lupus. Transfusion should remain rare in AHAI, but close contact with the transfusion service is necessary if it is to succeed. As for many autoimmune and/or systemic diseases, numerous types of treatment have been proposed but have not been validated in controlled multicenter studies. These are necessary to improve the management of these rare disorders.
自身免疫性溶血性贫血（AIHA）是在排除其他类型的溶血性贫血后，根据存在贫血（通常为大细胞性且程度不一）、网织红细胞增多以及直接和/或间接抗球蛋白试验阳性而确诊的。仅直接抗球蛋白试验阳性不足以诊断AIHA，在许多无贫血的患者中该试验可能为阳性，而在一些AIHA患者中可能为阴性。根据抗体活性的最佳温度，AIHA可分为两大类：温反应性自身抗体（通常为IgG），最佳活性温度约为37℃；冷反应性自身抗体，最佳活性温度为4℃（通常为IgM）。这种分类有助于指导检查和治疗的选择。尽管AIHA与多种其他疾病的关联往往是偶然的，但广泛报道称其与这些疾病有关。由于AIHA可能继发于病毒感染、淋巴系统恶性肿瘤或自身免疫性疾病（如狼疮），因此进行一套最少的有用检查是合适的。在AIHA中输血应尽量避免，但如果要进行输血，与输血服务部门密切联系是必要的。与许多自身免疫性和/或全身性疾病一样，已经提出了多种治疗方法，但尚未在对照多中心研究中得到验证。这些对照多中心研究对于改善这些罕见疾病的管理是必要的。
