一名患有冷抗体自身免疫性溶血性贫血儿童的血浆置换:病例报告。
Plasmapheresis in a child with cold antibody autoimmune hemolytic anemia: case report.
作者信息
Özdemir Zeynep Canan, Bör Özcan, Dinleyici Ener Çağrı, Kıral Eylem
机构信息
Department of Pediatrics, Division of Pediatric Hematology/Oncology, Eskişehir Osmangazi University Faculty of Medicine, Eskişehir, Turkey.
Department of Pediatrics, Division of Pediatric Intensive Care and Infectious Diseases, Eskişehir Osmangazi University Faculty of Medicine, Eskişehir, Turkey.
出版信息
Turk Pediatri Ars. 2017 Sep 1;52(3):169-172. doi: 10.5152/TurkPediatriArs.2017.2956. eCollection 2017 Sep.
Autoimmune hemolytic anemia is a picture of hemolysis which is caused by autoantibodies against red blood cell surface antigens. It is classified as primary, secondary or warm and cold autoimmune hemolytic anemia according to the temperature at which antibodies react. It is usually an acute and self-limiting condition. Here, we present a three-year-old male patient who presented with malaise, paleness, and dark-colored urine. His hemoglobin level was 5.8 g/dL, and increased indirect bilirubin and lactate dehydrogenase levels and decreased haptoglobulin and reticulocyte levels were noted. A direct Coombs test was positive using anti-C3. Four erythrocyte suspension transfusions were given because the anemia was life-threatening. High-dose steroids (30 mg/kg/ day, methylprednisolone) and intravenous immunoglobulin (1 g/kg/day, two days) treatments were unresponsive. Plasmapheresis was performed and no further transfusions were needed after plasmapheresis. Plasmapheresis treatment can be effective in children with cold type autoimmune hemolytic anemia.
自身免疫性溶血性贫血是一种由针对红细胞表面抗原的自身抗体引起的溶血表现。根据抗体反应的温度,它被分为原发性、继发性或温抗体型和冷抗体型自身免疫性溶血性贫血。它通常是一种急性自限性疾病。在此,我们报告一名3岁男性患者,他出现不适、面色苍白和深色尿。他的血红蛋白水平为5.8 g/dL,间接胆红素和乳酸脱氢酶水平升高,触珠蛋白和网织红细胞水平降低。使用抗C3进行直接抗人球蛋白试验呈阳性。由于贫血危及生命,给予了4次红细胞悬液输血。高剂量类固醇(30 mg/kg/天,甲泼尼龙)和静脉注射免疫球蛋白(1 g/kg/天,共2天)治疗无效。进行了血浆置换,血浆置换后无需进一步输血。血浆置换治疗对冷型自身免疫性溶血性贫血患儿可能有效。