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  • Title: Lung transplantation for pulmonary langerhans' cell histiocytosis: a multicenter analysis.
    Author: Dauriat G, Mal H, Thabut G, Mornex JF, Bertocchi M, Tronc F, Leroy-Ladurie F, Dartevelle P, Reynaud-Gaubert M, Thomas P, Pison C, Blin D, Stern M, Bonnette P, Dromer C, Velly JF, Brugière O, Lesèche G, Fournier M.
    Journal: Transplantation; 2006 Mar 15; 81(5):746-50. PubMed ID: 16534477.
    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> or =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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