Lung transplantation for pulmonary Langerhans' cell histiocytosis : A multicenter analysis - Université Claude Bernard Lyon 1 Accéder directement au contenu
Article Dans Une Revue Transplantation Année : 2006

Lung transplantation for pulmonary Langerhans' cell histiocytosis : A multicenter analysis

Résumé

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.

Dates et versions

hal-02666839 , version 1 (31-05-2020)

Identifiants

Citer

Gaelle Dauriat, Hervé Mal, Gabriel Thabut, Jean-François Mornex, Michelle Bertocchi, et al.. Lung transplantation for pulmonary Langerhans' cell histiocytosis : A multicenter analysis. Transplantation, 2006, 81 (5), pp.746-750. ⟨10.1097/01.tp.0000200304.64613.af⟩. ⟨hal-02666839⟩
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