PMID- 25118847
OWN - NLM
STAT- MEDLINE
DCOM- 20150612
LR  - 20140920
IS  - 2325-6621 (Electronic)
IS  - 2325-6621 (Linking)
VI  - 11
IP  - 7
DP  - 2014 Sep
TI  - Lung transplantation for advanced cystic lung disease due to nonamyloid kappa 
      light chain deposits.
PG  - 1025-31
LID - 10.1513/AnnalsATS.201404-137OC [doi]
AB  - RATIONALE: 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. OBJECTIVES: 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. MEASUREMENTS AND MAIN 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.
FAU - Hirschi, Sandrine
AU  - Hirschi S
AD  - 1 Service de Pneumologie, Groupe de Transplantation Pulmonaire, Nouvel Hopital 
      Civil, and.
FAU - Colombat, Magali
AU  - Colombat M
FAU - Kessler, Romain
AU  - Kessler R
FAU - Reynaud-Gaubert, Martine
AU  - Reynaud-Gaubert M
FAU - Stern, Marc
AU  - Stern M
FAU - Chenard, Marie Pierre
AU  - Chenard MP
FAU - Metivier, Anne-Cecile
AU  - Metivier AC
FAU - Jeung, Mi-Young
AU  - Jeung MY
FAU - Mal, Herve
AU  - Mal H
LA  - eng
PT  - Journal Article
PT  - Multicenter Study
PL  - United States
TA  - Ann Am Thorac Soc
JT  - Annals of the American Thoracic Society
JID - 101600811
RN  - 0 (Immunoglobulin kappa-Chains)
SB  - IM
MH  - Adult
MH  - Cystic Fibrosis/*diagnosis/mortality/*surgery
MH  - Female
MH  - Follow-Up Studies
MH  - Graft Rejection
MH  - Graft Survival
MH  - Humans
MH  - Immunoglobulin kappa-Chains/*metabolism
MH  - Kaplan-Meier Estimate
MH  - Lung Transplantation/*methods/mortality
MH  - Male
MH  - Respiratory Function Tests
MH  - Retrospective Studies
MH  - Risk Assessment
MH  - Sampling Studies
MH  - Severity of Illness Index
MH  - Survival Analysis
MH  - Treatment Outcome
MH  - Young Adult
OTO - NOTNLM
OT  - kappa-immunoglobulin light chain
OT  - lung diseases, obstructive
OT  - lung transplantation
OT  - respiratory insufficiency
EDAT- 2014/08/15 06:00
MHDA- 2015/06/13 06:00
CRDT- 2014/08/15 06:00
PHST- 2014/08/15 06:00 [entrez]
PHST- 2014/08/15 06:00 [pubmed]
PHST- 2015/06/13 06:00 [medline]
AID - 10.1513/AnnalsATS.201404-137OC [doi]
PST - ppublish
SO  - Ann Am Thorac Soc. 2014 Sep;11(7):1025-31. doi: 10.1513/AnnalsATS.201404-137OC.