title: Lung transplantation for advanced cystic lung disease due to nonamyloid kappa light chain deposits.
abstract: BACKGROUND
  Cystic lung light chain deposition disease (LCDD) is a severe and rare form of nonamyloid kappa light chain deposits localized in the lung, potentially leading to end-stage respiratory insufficiency.
  
  
  OBJECTIVE
  To assess the outcome after lung transplantation (LT) in this setting with particular attention to disease recurrence.
  
  
  METHODS
  We conducted a retrospective multicenter study of seven patients who underwent LT for cystic lung LCDD in France between September 1992 and June 2012 in five centers.
  
  
  RESULTS
  In total, five females and two males (mean age, 39.1 ± 5.3 yr) underwent one single LT or seven double LT (one retransplantation). Before LT, the patients showed a constant obstructive ventilatory pattern with low carbon monoxide diffusing capacity and resting hypoxemia. Lung computed tomography revealed widespread cysts with occasional micronodulations. No extrapulmonary disease or plasma cell neoplasm was detected. The serum-free kappa/lambda light chain ratio was increased in three cases. The median follow-up after LT was 56 months (range, 1-110 mo). Kaplan-Meier survival was 85.7, 85.7, and 64.3% at 1, 3, and 5 years, respectively. Three patients died from multiorgan failure (n = 1), chronic rejection (n = 1), and breast cancer (n = 1) at 23 days, 56 months, and 96 months, respectively. At the end of follow-up, no patients showed recurrence on imaging or histopathology.
  
  
  CONCLUSIONS
  This small case series confirms that cystic lung LCDD is a severe disease limited to the lung, affecting mostly young females. LT appears to be a good therapeutic option allowing for satisfactory long-term survival. We found no evidence of recurrence of the disease after LT.
authors.affiliation: 1 Service de Pneumologie, Groupe de Transplantation Pulmonaire, Nouvel Hôpital Civil, and.
authors.forename: Sandrine
authors.initials: S
authors.lastname: Hirschi
authors.forename: Magali
authors.initials: M
authors.lastname: Colombat
authors.forename: Romain
authors.initials: R
authors.lastname: Kessler
authors.forename: Martine
authors.initials: M
authors.lastname: Reynaud-Gaubert
authors.forename: Marc
authors.initials: M
authors.lastname: Stern
authors.forename: Marie Pierre
authors.initials: MP
authors.lastname: Chenard
authors.forename: Anne-Cécile
authors.initials: AC
authors.lastname: Métivier
authors.forename: Mi-Young
authors.initials: MY
authors.lastname: Jeung
authors.forename: Hervé
authors.initials: H
authors.lastname: Mal
mesh_terms: D000328:Adult; D003550:Cystic Fibrosis / Q000175:diagnosis* / Q000401:mortality / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006084:Graft Rejection; D006085:Graft Survival; D006801:Humans; D007145:Immunoglobulin kappa-Chains / Q000378:metabolism*; D053208:Kaplan-Meier Estimate; D016040:Lung Transplantation / Q000379:methods* / Q000401:mortality; D008297:Male; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D018570:Risk Assessment; D012494:Sampling Studies; D012720:Severity of Illness Index; D016019:Survival Analysis; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D007145:Immunoglobulin kappa-Chains
keywords: kappa-immunoglobulin light chain; lung diseases, obstructive; lung transplantation; respiratory insufficiency