Lung Transplantation for Pulmonary Langerhans’ Cell Histiocytosis: A Multicenter Analysis Gae ¨lle Dauriat, 1 Herve ´ Mal, 1,8 Gabriel Thabut, 1 Jean-Franc ¸ois Mornex, 2 Michelle Bertocchi, 2 Franc ¸ois Tronc, 2 Franc ¸ois Leroy-Ladurie, 3 Philippe Dartevelle, 3 Martine Reynaud-Gaubert, 4 Pascal Thomas, 4 Christophe Pison, 5 Dominique Blin, 5 Marc Stern, 6 Pierre Bonnette, 6 Claire Dromer, 7 Jean-Franc ¸ois Velly, 7 Olivier Brugie `re, 1 Guy Lese `che, 1 and Michel Fournier 1 Background. Lung transplantation (LT) may represent a therapeutic option in case of advanced pulmonary Langer- hans’ cell histiocytosis (PLCH). Little is known however about the characteristics of the patients considered for LT or its results. Methods. We conducted a retrospective multicenter study by questionnaire on 39 patients who underwent LT for end-stage PLCH at seven centers in France. Results. Of the 39 patients, 15 received single lung transplantation, 15 double lung transplantation and 9 heart-lung transplantation. At evaluation, extrapulmonary involvement was present in 31% of the patients, pulmonary hyperten- sion (PAPm25 mm Hg) was observed in 92% of cases and was moderate-to-severe (PAPm35 mm Hg) in 72.5%. The survival was 76.9% at 1 year, 63.6% at 2 years, 57.2% at 5 years, and 53.7% at 10 years. Recurrence of the disease occurred in eight cases (20.5%) with no impact on the survival rate. The sole risk factor for recurrence of the disease was the presence of preoperative extrapulmonary involvement. Conclusion. Severe pulmonary hypertension is a common feature in patients with end-stage PLCH. Given the good postransplant survival rate and despite a recurrence rate of the disease of approximately 20% after LT, we conclude that LT is a therapeutic option in this setting. Keywords: Lung transplantation, Histiocytosis, Langerhans cells, Eosinophilic granuloma. (Transplantation 2006;81: 746–750) L ung transplantation (LT) has gained widespread accep- tance as a therapeutic option in a selected number of pa- tients with end-stage lung disease refractory to other forms of treatment. Pulmonary Langerhans’ cell histiocytosis (PLCH), an uncommon cause of infiltrative lung disease that occurs principally in young adults, is one of these diseases which have become a potential indication for LT (1). This disease of unknown origin, which develops predominantly in adult cig- arettes smokers, belongs to the spectrum of Langerhans’ cell histiocytosis, diseases characterized by an uncontrolled pro- liferation and infiltration of various organs by Langerhans’ cells. In adult patients, the lung is the predominant target and is often the sole organ involved in the disease but other organs such as bone, skin, pituitary gland, and lymph nodes may also be involved. LT may be considered in a subgroup of PLCH patients who experience progressive respiratory impairment related to the extension of cystic lung destruction or to the development of severe pulmonary hypertension. To this date, several hundreds of PLCH patients have probably been trans- planted worldwide, but with the exception of some postoper- ative cases of recurrence of the disease that have been re- ported postLT (2–4), little information on LT in PLCH is available. Therefore, we designed a retrospective multicenter study to obtain information on LT in PLCH patients with special emphasis on posttransplant survival and frequency of recurrence of the disease. Another aim of the study was to assess the functional characteristics of the PLCH patients considered for LT and more particularly to describe their pul- monary hemodynamic status which is believed to play a cen- tral role in end-stage disease (5, 6). MATERIALS AND METHODS In August 2004, a standardized questionnaire was sent to each of the 11 adult lung transplantation centers in France to collect all cases of patients with PLCH who underwent LT in our country. In all cases, a firm diagnosis of PLCH based on the results of histological examination of the lung (preopera- tive lung biopsy, analysis of the explanted lung) was manda- tory. The questionnaire focused on several issues: smoking habit, extrathoracic involvement of Langerhans’ cell histiocy- tosis, results of preoperative pulmonary function tests and right heart catheterization, prevalence of pulmonary hyper- tension, modalities of the diagnosis of PLCH, type of LT [single lung transplantation (SLT), double lung transplanta- tion (DLT) or heart-lung transplantation (HLT)], intraoper- ative use of cardiopulmonary bypass, survival posttransplant, freedom from bronchiolitis obliterans syndrome (BOS), in- cidence of recurrence of PLCH, complications arising from the native lung in case of SLT, and incidence of malignancy. 1 Division of Pulmonary Medicine, Beaujon Hospital, Clichy, France. 2 Division of Pulmonary Medicine, Louis Pradel Hospital, Lyon, France. 3 Division of Thoracic Surgery, Marie-Lannelongue Hospital, Le Plessis- Robinson, France. 4 Division of Thoracic Surgery, Ste Marguerite Hospital, Marseille, France. 5 Division of Pulmonary Medicine and Thoracic Surgery, Albert Michalon Hospital, Grenoble, France. 6 Division of Pulmonary Medicine and Intensive Care Unit, Foch Hospital, Suresnes, France. 7 Division of Thoracic Surgery, Haut L’Eve ˆque Hospital, Bordeaux, France. 8 Address correspondence to: Herve ´ Mal, M.D., Service de Pneumologie et Re ´animation, Ho ˆpital Beaujon, 100 avenue du Ge ´ne ´ral Leclerc, 92110 Clichy, France. E-mail: herve.mal@bjn.ap-hop-paris.fr Received 26 May 2005. Revision requested 27 June 2005. Accepted 30 November 2005. Copyright © 2006 by Lippincott Williams & Wilkins ISSN 0041-1337/06/8105-746 DOI: 10.1097/01.tp.0000200304.64613.af 746 Transplantation • Volume 81, Number 5, March 15, 2006