PMID- 18483396
OWN - NLM
STAT- MEDLINE
DCOM- 20080930
LR  - 20211020
IS  - 1528-0020 (Electronic)
IS  - 0006-4971 (Print)
IS  - 0006-4971 (Linking)
VI  - 112
IP  - 5
DP  - 2008 Sep 1
TI  - Primary cystic lung light chain deposition disease: a clinicopathologic entity 
      derived from unmutated B cells with a stereotyped IGHV4-34/IGKV1 receptor.
PG  - 2004-12
LID - 10.1182/blood-2007-11-123596 [doi]
AB  - We have recently described a new form of light chain deposition disease (LCDD) 
      presenting as a severe cystic lung disorder requiring lung transplantation. There 
      was no bone marrow plasma cell proliferation. Because of the absence of disease 
      recurrence after bilateral lung transplantation and of serum-free light chain 
      ratio normalization after the procedure, we hypothesized that monoclonal light 
      chain synthesis occurred within the lung. The aim of this study was to look for 
      the monoclonal B-cell component in 3 patients with cystic lung LCDD. Histologic 
      examination of the explanted lungs showed diffuse nonamyloid kappa light chain 
      deposits associated with a mild lymphoid infiltrate composed of aggregates of 
      small CD20(+), CD5(-), CD10(-) B lymphocytes reminiscent of bronchus-associated 
      lymphoid tissue. Using polymerase chain reaction (PCR), we identified a dominant 
      B-cell clone in the lung in the 3 studied patients. The clonal expansion of each 
      patient shared an unmutated antigen receptor variable region sequence 
      characterized by the use of IGHV4-34 and IGKV1 subgroups with heavy and light 
      chain CDR3 sequences of more than 80% amino acid identity, a feature evocative of 
      an antigen-driven process. Combined with clinical and biologic data, our results 
      strongly argue for a new antigen-driven primary pulmonary lymphoproliferative 
      disorder.
FAU - Colombat, Magali
AU  - Colombat M
AD  - Assistance Publique des Hopitaux de Paris (APHP), Hopital Tenon, Service 
      d'anatomie pathologique, Paris, France.
FAU - Mal, Herve
AU  - Mal H
FAU - Copie-Bergman, Christiane
AU  - Copie-Bergman C
FAU - Diebold, Jacques
AU  - Diebold J
FAU - Damotte, Diane
AU  - Damotte D
FAU - Callard, Patrice
AU  - Callard P
FAU - Fournier, Michel
AU  - Fournier M
FAU - Farcet, Jean-Pierre
AU  - Farcet JP
FAU - Stern, Marc
AU  - Stern M
FAU - Delfau-Larue, Marie-Helene
AU  - Delfau-Larue MH
LA  - eng
PT  - Case Reports
PT  - Journal Article
DEP - 20080515
PL  - United States
TA  - Blood
JT  - Blood
JID - 7603509
RN  - 0 (Immunoglobulin Heavy Chains)
RN  - 0 (Immunoglobulin Light Chains)
RN  - 0 (Immunoglobulin Variable Region)
RN  - 0 (Immunoglobulin kappa-Chains)
RN  - 9007-49-2 (DNA)
SB  - IM
MH  - Adult
MH  - Amino Acid Sequence
MH  - B-Lymphocytes/*immunology/*pathology
MH  - Base Sequence
MH  - Cysts/genetics/*immunology/*pathology/surgery
MH  - DNA/genetics
MH  - Female
MH  - Genes, Immunoglobulin Heavy Chain
MH  - Genes, Immunoglobulin Light Chain
MH  - Humans
MH  - Immunoglobulin Heavy Chains/genetics/metabolism
MH  - Immunoglobulin Light Chains/genetics/*metabolism
MH  - Immunoglobulin Variable Region/genetics/metabolism
MH  - Immunoglobulin kappa-Chains/genetics/metabolism
MH  - Lung Diseases/genetics/*immunology/*pathology/surgery
MH  - Lung Transplantation
MH  - Molecular Sequence Data
MH  - Paraproteinemias/genetics/*immunology/*pathology/surgery
PMC - PMC2572763
MID - HALMS326865
OID - NLM: HALMS326865
EDAT- 2008/05/17 09:00
MHDA- 2008/10/01 09:00
PMCR- 2008/10/28
CRDT- 2008/05/17 09:00
PHST- 2008/05/17 09:00 [pubmed]
PHST- 2008/10/01 09:00 [medline]
PHST- 2008/05/17 09:00 [entrez]
PHST- 2008/10/28 00:00 [pmc-release]
AID - S0006-4971(20)49635-1 [pii]
AID - 10.1182/blood-2007-11-123596 [doi]
PST - ppublish
SO  - Blood. 2008 Sep 1;112(5):2004-12. doi: 10.1182/blood-2007-11-123596. Epub 2008 
      May 15.