Lung transplantation for pulmonary langerhans' cell histiocytosis: a multicenter analysis

Transplantation. 2006 Mar 15;81(5):746-50. doi: 10.1097/


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.

Publication types

  • Multicenter Study

MeSH terms

  • Adolescent
  • Adult
  • Female
  • Heart-Lung Transplantation / mortality
  • Histiocytosis, Langerhans-Cell / diagnosis*
  • Histiocytosis, Langerhans-Cell / mortality
  • Histiocytosis, Langerhans-Cell / surgery*
  • Humans
  • Hypertension, Pulmonary / diagnosis*
  • Hypertension, Pulmonary / mortality
  • Hypertension, Pulmonary / surgery
  • Lung Diseases / diagnosis*
  • Lung Diseases / mortality
  • Lung Diseases / surgery*
  • Lung Transplantation* / mortality
  • Male
  • Middle Aged
  • Recurrence
  • Retrospective Studies
  • Treatment Outcome