Lung transplantation for lymphangioleiomyomatosis: the European experience.
BACKGROUND
Lung transplantation has been accepted widely as therapy for end-stage pulmonary lymphangioleiomyomatosis (LAM); however, single-center and national experience is limited due to the rarity of LAM.


METHODS
We report the recent European experience of lung transplantation for LAM. A self-administered questionnaire was distributed to 30 European lung transplant centers to evaluate patients who underwent primary lung transplantation for LAM (1997 to 2007).


RESULTS
Seventy percent of centers responded to the questionnaire. A total of 61 lung transplants were undertaken in women only, with mean age at transplant 41.3 years (SD 5.1). Centers performed a median of 2 (0 to 9) transplant operations. Severe pleural adhesions were the most common intra-operative complication. Early deaths (N = 6) were due to primary graft or multiple-organ failure or sepsis. Twelve recipients were diagnosed with bronchiolitis obliterans syndrome at a median of 20 months (range 10 to 86 months) post-transplant. LAM-related complications included renal angiomyolipoma and pneumothorax in the native lung. Recurrence of LAM occurred in 4 recipients. As of December 2007, actuarial Kaplan-Meier survival was 79% at 1 year and 73% at 3 years post-transplant.


CONCLUSIONS
Post-transplant outcome for pulmonary LAM in the recent era appears to have improved compared with the previous era. LAM-related complications remain common, but recurrence of LAM in the allograft is rare.
Benden
Christian
C
Division of Pulmonary Medicine, University Hospital Zurich, Zurich, Switzerland.
Rea
Federico
F
Behr
Jürgen
J
Corris
Paul A
PA
Reynaud-Gaubert
Martine
M
Stern
Marc
M
Speich
Rudolf
R
Boehler
Annette
A
D000328:Adult; D002423:Cause of Death; D004359:Drug Therapy, Combination; D005060:Europe; D005080:Exercise Test; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007431:Intraoperative Complications / Q000145:classification / Q000453:epidemiology; D008171:Lung Diseases / Q000453:epidemiology; D008175:Lung Neoplasms / Q000601:surgery*; D016040:Lung Transplantation / Q000401:mortality / Q000502:physiology*; D008204:Lymphangiosarcoma / Q000601:surgery*; D012129:Respiratory Function Tests; D011795:Surveys and Questionnaires; D016019:Survival Analysis; D000267:Tissue Adhesions / Q000453:epidemiology; D016896:Treatment Outcome
D007166:Immunosuppressive Agents