Shvidel L, Shtalrid M, Duek A, Haran M, Berrebi A, Sigler E
Hematology Institute, Kaplan Medical Center, Rehovot, affiliated to Hebrew University and Hadassah Medical School, Jerusalem, Israel.
Int J Lab Hematol. 2008 Dec;30(6):494-8. doi: 10.1111/j.1751-553X.2007.00985.x.
Direct antiglobulin test (DAT) with only complement detected on red blood cells is a rare laboratory finding, and its significance in the setting of warm autoimmune hemolytic anemia (AIHA) is controversial. During 2 years (2003-2004) 277 patients with positive DAT were recorded in the blood bank registries, 17 of them had DAT reactive with C3 alone with no cold agglutinin or other nonimmune causes for hemolysis diagnosed. Red cell eluate disclosed small amounts of IgG in two patients. In nine patients no signs of clinical hemolysis were found, however, all these patients had underlying conditions that are known to be associated with red cells autoantibodies (autoimmune disorder or malignancy). Eight patients developed AIHA, seven of them with severe hemolysis. Three patients had idiopathic AIHA, and the others have been diagnosed with infectious, lymphoproliferative and autoimmune disorders. In two patients with acute infection the hemolytic process spontaneously resolved, three responded to corticosteroid therapy, while three patients were refractory to two lines of drug therapy and underwent splenectomy. Reticulocytopenia was found in four patients. Our results emphasize that AIHA with DAT reactive with complement alone is a rare disorder and might be accompanied by severe, refractory to conventional treatment and life-threatening hemolysis.
直接抗球蛋白试验（DAT）仅在红细胞上检测到补体是一种罕见的实验室检查结果，其在温抗体型自身免疫性溶血性贫血（AIHA）中的意义存在争议。在2年（2003 - 2004年）期间，血库登记记录了277例DAT阳性患者，其中17例DAT仅与C3反应，未诊断出冷凝集素或其他非免疫性溶血原因。红细胞洗脱液在两名患者中发现少量IgG。9例患者未发现临床溶血迹象，然而，所有这些患者都有已知与红细胞自身抗体相关的基础疾病（自身免疫性疾病或恶性肿瘤）。8例患者发生AIHA，其中7例有严重溶血。3例为特发性AIHA，其他患者被诊断为感染性、淋巴增殖性和自身免疫性疾病。2例急性感染患者的溶血过程自发缓解，3例对皮质类固醇治疗有反应，而3例患者对两线药物治疗无效并接受了脾切除术。4例患者出现网织红细胞减少。我们的结果强调，仅与补体反应的DAT的AIHA是一种罕见疾病，可能伴有严重的、对传统治疗难治且危及生命的溶血。
