Case report Survival of Burkholderia cepacia sepsis following lung transplantation in recipients with cystic ¢brosis E.F. Nash 1,2 , A. Coonar 1,3 , R. Kremer 1,3 , E. Tullis 2,4 , M. Hutcheon 1 , L.G. Singer 1 , S. Keshavjee 1,3 , C. Chaparro 1,2 1 Toronto Lung Transplant Program, University Health Network, University of Toronto, 2 Toronto Adult CF Clinic, St Michael’s Hospital, University of Toronto, 3 Department of Surgery, Division of Thoracic Surgery, University of Toronto, Toronto, Ontario, Canada, 4 Li Ka Shing Knowledge Institute, Toronto, Ontario, Canada E.F. Nash, A. Coonar, R. Kremer, E.Tullis, M. Hutcheon, L.G. Singer, S. Keshavjee, C. Chaparro. Survival of Burkholderia cepacia sepsis following lung transplantation in recipients with cystic ¢brosis. Transpl Infect Dis 2010: 12: 551^554. All rights reserved Abstract: Cystic ¢brosis (CF) lung transplant recipients infected with Burkholderia cenocepacia have a worse survival rate after lung transplantation than those who are not infected with this organism.The decreased survival is predominantly due to recurrent B. cenocepacia infection, with the majority of a¡ected recipients succumbing within 3 months after transplant. B. cepacia complex (BCC) sepsis is one of the de¢ning criteria for cepacia syndrome, an almost universally fatal necrotizing pneumonic illness.We report 2 CF patients who were long- term survivors of B. cenocepacia sepsis after lung transplantation.The aim of this report is to demonstrate that, although survival of B. cenocepacia sepsis after lung transplantation is extremely uncommon, with aggressive multidisciplinary management, long-term survival remains a realistic objective.                                                                                                 Burkholderia cepacia complex (BCC) refers to a group of at least 9 closely related bacterial species (previously referred to as ‘genomovars’) that can cause pulmonary infection in people with cystic ¢brosis (CF). Burkholderia multivorans (formerly ‘genomovar II’) and Burkholderia cenocepacia (for- merly ‘genomovar III’) are the 2 most commonly isolated spe- cies, but all the species within the BCC can cause chronic CF airway infection (1). B. cenocepacia infection has a major adverse impact on survival of CF patients after lung trans- plantation (2) and is a contraindication to lung transplanta- tion in many centers (3). In the pre-transplant setting, BCC infection results in heterogeneous clinical outcomes, rang- ing from asymptomatic carriage to the most extreme manifestation, termed cepacia syndrome (4). Cepacia syn- drome is characterized by a rapidly progressive necrotizing pneumonia with an almost universally fatal outcome, and BCC sepsis is one of the de¢ning criteria for the diagnosis. Survival of CF patients from cepacia syndrome has been re- ported infrequently (5^7), but survival of these patients with cepacia syndrome after lung transplantation seems even less likely, because of the intensive immunosuppression re- quired to prevent organ rejection. We present 2 cases of long-term survival after B. cenocepacia sepsis (cepacia syndrome) after lung trans- plantation. The aim of this case report is to demonstrate that, although mortality is very high in this situation, with aggressive multidisciplinary management, long-term sur- vival remains a realistic objective. Case reports Patient 1 Patient 1was diagnosed with CFat 5 months of age, and he became infected with the ET12 strain of B. cenocepacia r 2010 John Wiley & Sons A/S Transplant Infectious Disease . ISSN 1398-2273 Key words: cystic fibrosis; Burkholderia cepacia complex; Burkholderia cenocepacia; cepacia syndrome; lung transplantation; sepsis; survival Correspondence to: Dr Edward F. Nash, MRCP (UK), Consultant in Respiratory Medicine and Cystic Fibrosis, West Midlands Adult Cystic Fibrosis Centre, Heartlands Hospital, Bordesley Green East, Birmingham B9 5SS, UK Tel: 1 01144 1 214 241 669 Fax: 1 01144 1 214 241 661 E-mail: edward.nash@heartofengland.nhs.uk Received 8 November 2009, revised 8 March 2010, accepted for publication 22 March 2010 DOI: 10.1111/j.1399-3062.2010.00525.x Transpl Infect Dis 2010: 12: 551–554 551