Ulvestad E
Department of Microbiology and Immunology, The Gade Institute, Haukeland Hospital, Bergen, Norway.
Eur J Haematol. 1998 Feb;60(2):93-100. doi: 10.1111/j.1600-0609.1998.tb01004.x.
A patient with classical cold agglutinin disease initially experienced haemolytic episodes during cold exposure. However, with advancing disease cold-induced haemolysis ceased and was substituted with a haemolytic disposition at elevated body temperatures. To investigate this paradoxical development of disease manifestations, we performed a clinical and immunological study. Our results indicate that the patient's complement system became exhausted during the later phase of his disease, probably due to continual consumption of complement components. Initially, the patient had slightly decreased C4 concentrations and moderately reduced total haemolytic activity (CH50). Later C4 fell to undetectable levels and CH50 declined to zero. The increased haemolytic activity experienced during febrile episodes is probably due to a cold agglutinin with a high thermal amplitude, combined with enhanced synthesis of complement molecules during the acute phase response. Although C4 concentrations never increased to detectable levels during infections or inflammations an acute phase reaction was determined each time, as evidenced by increased concentrations of CRP. By reconstituting the patient's serum with active complement from donor serum or plasma, increased haemolytic activity was observed. These results indicate that some patients with cold agglutinin disease may experience deleterious haemolytic consequences if transfused with plasma-containing blood products.
一名典型冷凝集素病患者最初在寒冷暴露期间出现溶血发作。然而，随着病情进展，冷诱导的溶血停止，取而代之的是在体温升高时出现溶血倾向。为了研究疾病表现的这种矛盾发展，我们进行了一项临床和免疫学研究。我们的结果表明，该患者的补体系统在疾病后期耗尽，可能是由于补体成分的持续消耗。最初，患者的C4浓度略有下降，总溶血活性（CH50）中度降低。后来C4降至无法检测的水平，CH50降至零。发热发作期间溶血活性增加可能是由于具有高热幅度的冷凝集素，以及急性期反应期间补体分子合成增强。尽管在感染或炎症期间C4浓度从未增加到可检测水平，但每次都确定有急性期反应，如CRP浓度增加所证明。通过用供体血清或血浆中的活性补体重建患者的血清，观察到溶血活性增加。这些结果表明，一些冷凝集素病患者如果输注含血浆的血液制品，可能会出现有害的溶血后果。
