PMID- 16399989
OWN - NLM
STAT- MEDLINE
DCOM- 20061003
LR  - 20060324
IS  - 1073-449X (Print)
IS  - 1073-449X (Linking)
VI  - 173
IP  - 7
DP  - 2006 Apr 1
TI  - Pulmonary cystic disorder related to light chain deposition disease.
PG  - 777-80
AB  - Light chain deposition disease (LCDD) is a rare disorder that very uncommonly 
      affects the lung. We report three cases of severe cystic pulmonary LCDD leading 
      to lung transplantation. Such a presentation has never been previously reported. 
      The three patients present with a progressive obstructive pulmonary pattern 
      associated with numerous cysts diffusely distributed in both lungs. The disease 
      was histologically characterized by non-amyloid amorphous deposits in the 
      alveolar walls, the small airways and the vessels. It was associated with 
      emphysematous-like changes and small airway dilation. Monotypic kappa light chain 
      fixation was demonstrated on the abnormal deposits and along the basement 
      membranes. Electron microscopy revealed coarsely granular electron-dense deposits 
      in the same localizations. Mild extrapulmonary deposits were found in salivary 
      glands in one patient. No immunoproliferative disorder was identified. We 
      conclude that LCDD may primarily affect the lung, present as a pulmonary cystic 
      disorder, and lead to severe respiratory insufficiency.
FAU - Colombat, Magali
AU  - Colombat M
AD  - Service d'Anatomie Pathologique, Hopital Beaujon, Clichy, France. 
      colombatm@yahoo.fr
FAU - Stern, Marc
AU  - Stern M
FAU - Groussard, Odile
AU  - Groussard O
FAU - Droz, Dominique
AU  - Droz D
FAU - Brauner, Michel
AU  - Brauner M
FAU - Valeyre, Dominique
AU  - Valeyre D
FAU - Mal, Herve
AU  - Mal H
FAU - Taille, Camille
AU  - Taille C
FAU - Monnet, Isabelle
AU  - Monnet I
FAU - Fournier, Michel
AU  - Fournier M
FAU - Herson, Serge
AU  - Herson S
FAU - Danel, Claire
AU  - Danel C
LA  - eng
PT  - Case Reports
PT  - Journal Article
DEP - 20060106
PL  - United States
TA  - Am J Respir Crit Care Med
JT  - American journal of respiratory and critical care medicine
JID - 9421642
RN  - 0 (Immunoglobulin Light Chains)
SB  - IM
MH  - Adult
MH  - Biopsy
MH  - Cystic Fibrosis/diagnosis/*etiology/surgery
MH  - Diagnosis, Differential
MH  - Fatal Outcome
MH  - Female
MH  - Humans
MH  - Hypergammaglobulinemia/*complications/diagnosis/metabolism
MH  - Immunoglobulin Light Chains/*metabolism/ultrastructure
MH  - Lung Transplantation
MH  - Male
MH  - Microscopy, Electron
MH  - Tomography, X-Ray Computed
EDAT- 2006/01/10 09:00
MHDA- 2006/10/04 09:00
CRDT- 2006/01/10 09:00
PHST- 2006/01/10 09:00 [pubmed]
PHST- 2006/10/04 09:00 [medline]
PHST- 2006/01/10 09:00 [entrez]
AID - 200510-1620CR [pii]
AID - 10.1164/rccm.200510-1620CR [doi]
PST - ppublish
SO  - Am J Respir Crit Care Med. 2006 Apr 1;173(7):777-80. doi: 
      10.1164/rccm.200510-1620CR. Epub 2006 Jan 6.