Sweeting Claire A, Kelleher Noel, Mahmood Nilofer, Unsworth-White Jonathan, Weatherall Mike, Webb Gerry
Department of Clinical Perfusion and Research, Derriford Hospital, Plymouth, UK.
Perfusion. 2004 Nov;19(6):381-3. doi: 10.1191/0267659104pf774cr.
Waldenstrom's Macroglobulinaemia (WM) is a rare haematological lymphoma that causes numerous haemostatic complications due to the elevated levels of immunoglobulin M (IgM) circulating in the blood. These complications, such as hyperviscosity syndrome, may be exacerbated by the physiological effects of cardiopulmonary bypass (CPB). In this case study, a 45-year-old male suffering from WM underwent an emergency aortic valve replacement, closure of an atrio-ventricular fistula and mitral valve repair. He was found to have an elevated blood viscosity, anaemia and hypervolaemia prior to surgery. These complications remained a problem during CPB, leading to a large circulating volume, but a low haemoglobin, requiring haemofiltration and blood transfusions whilst limiting any further rise in blood viscosity. The situation was further compounded by the presence of electrolyte disturbances. It was concluded that a careful balance between blood conservation techniques and temperature management was required to reduce hyperviscosity and anaemia, but maintain organ protection. In future, given more time, modern plasmapheresis techniques could be used for acute management of WM during CPB.
华氏巨球蛋白血症(WM)是一种罕见的血液系统淋巴瘤,由于血液中循环的免疫球蛋白M(IgM)水平升高,会引发多种止血并发症。这些并发症,如高黏滞综合征,可能会因体外循环(CPB)的生理效应而加重。在本病例研究中,一名患有WM的45岁男性接受了紧急主动脉瓣置换、房室瘘闭合和二尖瓣修复手术。术前发现他血液黏度升高、贫血且血容量过多。这些并发症在CPB期间仍然是个问题,导致循环血量很大,但血红蛋白水平低,需要进行血液滤过和输血,同时限制血液黏度的进一步升高。电解质紊乱的存在使情况更加复杂。得出的结论是,需要在血液保护技术和体温管理之间谨慎平衡,以降低高黏滞度和贫血,但要维持器官保护。未来,如果有更多时间,现代血浆置换技术可用于CPB期间WM的急性处理。