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


CONCLUSIONS
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.
Dauriat
Gaëlle
G
Division of Pulmonary Medicine, Beaujon Hospital, Clichy, France. 2 Division of Pulmonary Medicine, Louis Pradel Hospital, Lyon, France.
Mal
Hervé
H
Thabut
Gabriel
G
Mornex
Jean-François
JF
Bertocchi
Michelle
M
Tronc
François
F
Leroy-Ladurie
François
F
Dartevelle
Philippe
P
Reynaud-Gaubert
Martine
M
Thomas
Pascal
P
Pison
Christophe
C
Blin
Dominique
D
Stern
Marc
M
Bonnette
Pierre
P
Dromer
Claire
C
Velly
Jean-François
JF
Brugière
Olivier
O
Lesèche
Guy
G
Fournier
Michel
M
D000293:Adolescent; D000328:Adult; D005260:Female; D016041:Heart-Lung Transplantation / Q000401:mortality; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis* / Q000401:mortality / Q000601:surgery*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000401:mortality / Q000601:surgery; D008171:Lung Diseases / Q000175:diagnosis* / Q000401:mortality / Q000601:surgery*; D016040:Lung Transplantation / Q000401:mortality; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D012189:Retrospective Studies; D016896:Treatment Outcome