Ichikawa Y, Tanaka C, Ohya N, Hida M
St. Marianna University School of Medicine, Institute of Medical Science.
Nihon Rinsho. 1996 Sep;54(9):2528-33.
Hemolytic anemia observed in patients with SLE include autoimmune hemolytic anemia (AIHA) and thrombotic microangiopathic hemolytic anemia (TMHA). AIHA is caused by anti-erythrocyte antibody, which can be detected by direct Coombs' test. Recently, anti-phospholipid antibody draw attention as a cause of AIHA in SLE. TMHA include thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). Patients with TTP show CNS symptoms and renal damage, while those with HUS develop renal insufficiency, but show minimal CNS symptoms. Clinical findings of TMHA are fever, hemolytic anemia, thrombocytopenia, CNS symptoms, and renal insufficiency, which are quite same as the findings of SLE itself. TMHA should be considered for differential diagnosis in acute exacerbation of SLE.
系统性红斑狼疮(SLE)患者中观察到的溶血性贫血包括自身免疫性溶血性贫血(AIHA)和血栓性微血管病性溶血性贫血(TMHA)。AIHA由抗红细胞抗体引起,可通过直接抗人球蛋白试验检测到。最近,抗磷脂抗体作为SLE中AIHA的一个病因受到关注。TMHA包括血栓性血小板减少性紫癜(TTP)和溶血性尿毒症综合征(HUS)。TTP患者表现出中枢神经系统症状和肾脏损害,而HUS患者则出现肾功能不全,但中枢神经系统症状轻微。TMHA的临床表现为发热、溶血性贫血、血小板减少、中枢神经系统症状和肾功能不全,这些与SLE本身的表现非常相似。在SLE急性加重时应考虑TMHA以进行鉴别诊断。