Fink F M, Dengg K, Kilga-Nogler S, Schönitzer D, Berger H
Department of Paediatrics, School of Medicine, University of Innsbruck, Austria.
Eur J Pediatr. 1992 Jun;151(6):435-7. doi: 10.1007/BF01959358.
Acute cold haemagglutinin disease, most commonly associated with underlying mycoplasma infection, is rare in children. A 3-year-old girl who developed this auto-immune disease under intensive cytotoxic treatment for rhabdomyosarcoma is presented. Clinically, a livedo reticularis skin pattern upon exposure to cold which was reversible at room temperature and a spontaneous red cell agglutination of blood samples in vitro led to the diagnosis. Together with bronchopneumonia the girl developed hyper-IgM, high antibody titres against Mycoplasma pneumoniae, as well as high titres of cold agglutinins. Laboratory signs of mild intravascular haemolysis were found. Positive direct antiglobulin test resulted from coating of red cells with C3d and C4. Three different antibodies were identified in serum: nonspecific cold agglutinins without complement activation, anti-I specific cold agglutinins with complement activation, as well as a weak biphasic Donath-Landsteiner haemolysin. Under antibiotic treatment and a short course of predisolone the clinical course was mild.
急性冷凝集素病在儿童中较为罕见，最常见于潜在的支原体感染。本文报告了一名3岁女童，她在接受横纹肌肉瘤强化细胞毒性治疗时患上了这种自身免疫性疾病。临床上，患儿暴露于寒冷环境时出现网状青斑皮肤表现，在室温下可逆转，且体外血液样本出现自发红细胞凝集，据此做出诊断。该女童除支气管肺炎外，还出现了高IgM、抗肺炎支原体抗体滴度升高以及冷凝集素滴度升高。实验室检查发现轻度血管内溶血迹象。直接抗球蛋白试验阳性是由于红细胞被C3d和C4包被。血清中鉴定出三种不同抗体：无补体激活的非特异性冷凝集素、有补体激活的抗I特异性冷凝集素以及弱阳性双相Donath-Landsteiner溶血素。在抗生素治疗和短期泼尼松龙治疗下，临床病程较为轻微。
