Grainger J D, Makar Y, McManus A, Wynn R
Manchester Children's Hospital, Pendlebury, Manchester, UK.
Transfus Med. 2001 Feb;11(1):55-7. doi: 10.1046/j.1365-3148.2001.00278.x.
We report the case of a 1-year-old girl with newly diagnosed beta-thalassaemia major. Following an initial blood transfusion with phenotypically matched blood, she developed a haemolytic anaemia which progressed with subsequent transfusions. The Direct Antiglobulin test (DAT) was strongly positive with C3d and weakly with IgG. The only free antibodies detected were a weak anti-H and a weak cold auto-antibody, which did not exhibit a wide thermal range. The indirect Donath-Landsteiner and Ham's tests were negative. There was no sustained clinical response to steroids, immunoglobulin infusions or splenectomy. An HLA identical sibling donor was available for allogeneic bone marrow transplantation (BMT) and the haemolysis resolved during the immunosuppressive transplant conditioning. Such hyperhaemolysis without significant red cell alloantibodies has previously been reported in patients with sickle cell anaemia, but only rarely in patients with beta-thalassaemia major.
我们报告了一例新诊断为重型β地中海贫血的1岁女童病例。在首次输注表型匹配的血液后，她发生了溶血性贫血，且在随后的输血过程中病情进展。直接抗人球蛋白试验（DAT）中C3d呈强阳性，IgG呈弱阳性。检测到的唯一游离抗体是弱抗-H和弱冷自身抗体，其热反应范围不宽。间接Donath-Landsteiner试验和Ham试验均为阴性。使用类固醇、输注免疫球蛋白或脾切除术均未产生持续的临床反应。有一位HLA配型相同的同胞供体可用于异基因骨髓移植（BMT），并且在免疫抑制性移植预处理期间溶血得到缓解。这种无明显红细胞同种抗体的高溶血现象先前在镰状细胞贫血患者中已有报道，但在重型β地中海贫血患者中极为罕见。
