Tjønnfjord Eirik, Vengen Øystein A, Berentsen Sigbjørn, Tjønnfjord Geir Erland
Hematology and Oncology, Kalnes Sykehus Østfold, Kalnes, Grålum, Norway.
Department of Cardiothoracic Surgery, Oslo Universitetssykehus, Oslo, Norway.
BMJ Case Rep. 2017 May 9;2017:bcr-2016-219066. doi: 10.1136/bcr-2016-219066.
Primary chronic cold agglutinin disease (CAD) is an autoimmune haemolytic anaemia in which a specific bone marrow lymphoproliferative disorder causes production of cold agglutinins (CA). Binding of CA to erythrocyte surface antigens results in a predominantly extravascular haemolysis that is entirely complement dependent. Because of complement activation, exacerbations are common during febrile infections, trauma or major surgery. Involvement of the terminal complement pathway with C5-mediated intravascular haemolysis is probably not prominent in stable disease but is supposed to be of importance in exacerbations following acute phase reaction.We report on a patient with CAD prone to exacerbation of haemolysis during acute phase reactions who was scheduled for cardiac surgery. To prevent her having an exacerbation of haemolysis, we chose to treat her prophylactically with eculizumab along with the usual perioperative precautions. Aortic valve replacement was undertaken with full cardiopulmonary bypass at normothermia. The procedure was successful; no exacerbation of haemolysis was observed, and transfusion requirements did not exceed what could be expected.
原发性慢性冷凝集素病（CAD）是一种自身免疫性溶血性贫血，其中特定的骨髓淋巴增殖性疾病导致冷凝集素（CA）的产生。CA与红细胞表面抗原的结合导致主要为血管外溶血，这完全依赖补体。由于补体激活，在发热性感染、创伤或大手术期间病情加重很常见。在稳定疾病中，终末补体途径与C5介导的血管内溶血的参与可能不突出，但在急性期反应后的病情加重中被认为很重要。我们报告了一名在急性期反应期间易发生溶血加重的CAD患者，该患者计划进行心脏手术。为防止她发生溶血加重，我们选择用依库珠单抗对她进行预防性治疗，并采取了常规的围手术期预防措施。在常温下进行了全心肺转流的主动脉瓣置换术。手术成功；未观察到溶血加重，输血需求未超过预期。
