Transplantation. 81(5):746-750, MARCH 15TH, 2006

DOI: 10.1097/01.tp.0000200304.64613.af

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PMID: 16534477

Issn Print: 0041-1337

Publication Date: March 15th, 2006


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Lung Transplantation for Pulmonary Langerhans' Cell Histiocytosis: A Multicenter Analysis

Gaëlle Dauriat;Hervé Mal;Gabriel Thabut;Jean-François Mornex;Michelle Bertocchi;François Tronc;François Leroy-Ladurie;Philippe Dartevelle;Martine Reynaud-Gaubert;Pascal Thomas;Christophe Pison;Dominique Blin;Marc Stern;Pierre Bonnette;Claire Dromer;Jean-François Velly;Olivier Brugière;Guy Lesèche;Michel Fournier;

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Abstract

Background.Lung transplantation (LT) may represent a therapeutic option in case of advanced pulmonary Langerhans' 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 hypertension (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.

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