Hillyer C D, Schwenn M R, Fulton D R, Meissner H C, Berkman E M
Department of Medicine, New England Medical Center, Boston, Massachusetts.
Transfusion. 1990 Oct;30(8):738-40. doi: 10.1046/j.1537-2995.1990.30891020336.x.
A 3-year-old boy presented with the fever, conjunctivitis, rash, and lymphadenopathy diagnostic of Kawasaki disease. Treatment with antibiotics, aspirin, and intravenous immunoglobulin was instituted. The hematocrit decreased from 35 percent on admission to 11 percent by hospital Day 10, and the white cell count had increased from 13.7 to 42 x 10(3) per microL, and the patient had a leukoerythroblastic blood smear. The direct antiglobulin test demonstrated IgG but not complement on the red cell (RBC) surface. An acid eluate reacted (titer of 4) with all panel cells in the antiglobulin phase. Intravenous immunoglobulin from the same lot used for treatment did not contain antibody that reacted with the patient's group O RBCs or a panel of group O RBCs, but did contain IgG anti-A and -B (titer of 4). The patient received a transfusion and was given methylprednisone. The direct antiglobulin test and acid eluate were negative 4 days later. The patient had an uneventful recovery. The distinction between antibody-mediated hemolytic anemia and autoimmune hemolytic anemia is important in the treatment of this disease.
一名3岁男孩因发热、结膜炎、皮疹和淋巴结病就诊,诊断为川崎病。给予抗生素、阿司匹林和静脉注射免疫球蛋白治疗。入院时血细胞比容为35%,到住院第10天时降至11%,白细胞计数从13.7×10³/μL增至42×10³/μL,患者血涂片显示有幼粒-幼红细胞象。直接抗球蛋白试验显示红细胞(RBC)表面有IgG但无补体。酸洗脱液在抗球蛋白试验阶段与所有谱细胞发生反应(效价为4)。用于治疗的同一批次静脉注射免疫球蛋白不含与患者O型RBC或一组O型RBC发生反应的抗体,但确实含有IgG抗A和抗B(效价为4)。患者接受了输血并给予甲泼尼龙。4天后直接抗球蛋白试验和酸洗脱液均为阴性。患者康复顺利。在该疾病的治疗中,区分抗体介导的溶血性贫血和自身免疫性溶血性贫血很重要。