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Manon Levêque,1 Nadim Cassir,2 Fanny Mathias,3 Cindy Fevre,4 Florence Daviet,5 Julien Bermudez,1 Geoffrey Brioude,6 Florence Peyron,3 Martine Reynaud-Gaubert,1 Benjamin Coiffard1 1Department of Respiratory Medicine and Lung Transplantation, APHM, Aix Marseille University, Hôpital Nord, Marseille, France; 2Department of Infectious Disease, APHM, IHU Méditerranée Infection, Aix-Marseille University, Marseille, France; 3Department of Pharmacy, APHM, Aix Marseille University, Hôpital Nord, Marseille, France; 4Research and Development, Pherecydes Pharma, Romainville, France; 5Intensive Care Medicine, APHM, Aix Marseille University, Hôpital Nord, Marseille, France; 6Department of Thoracic Surgery and Lung Transplantation, APHM, Aix Marseille University, Hôpital Nord, Marseille, FranceCorrespondence: Benjamin Coiffard, Department of Respiratory Medicine and Lung Transplantation, APHM, CHU Nord, Chemin des Bourrely, Marseille, 13915, France, Tel +33491966133, Email benjamin.coiffard@ap-hm.frAbstract: Recipients transplanted for bronchiectasis in the context of a primary immune deficiency, such as common variable immunodeficiency, are at a high risk of severe infection in post-transplantation leading to poorer long-term outcomes than other transplant indications. In this report, we present a fatal case due to chronic Pseudomonas aeruginosa bronchopulmonary infection in a lung transplant recipient with common variable immunodeficiency despite successful eradication of an extensively drug-resistant (XDR) strain with IgM/IgA-enriched immunoglobulins and bacteriophage therapy. The fatal evolution despite a drastic adaptation of the immunosuppressive regimen and the maximal antibiotic therapy strategy raises the question of the contraindication of lung transplantation in such a context of primary immunodeficiency.Keywords: primary immunodeficiency diseases, lung transplantation, Pseudomonas aeruginosa, phage therapy, immunoglobulin therapy