PMID- 30967366
OWN - NLM
STAT- MEDLINE
DCOM- 20200109
LR  - 20210202
IS  - 1528-0020 (Electronic)
IS  - 0006-4971 (Linking)
VI  - 133
IP  - 26
DP  - 2019 Jun 27
TI  - Proteomic evidence of specific IGKV1-8 association with cystic lung light chain 
      deposition disease.
PG  - 2741-2744
LID - 10.1182/blood.2019898577 [doi]
AB  - We previously reported a new form of light chain deposition disease (LCDD) 
      presenting as diffuse cystic lung disorder that differs from the usual systemic 
      form with respect to patient age, the male/female ratio, the involved organs, and 
      the hematologic characteristics. We also demonstrated that the light chains were 
      produced by an intrapulmonary B-cell clone and that this clone shared a 
      stereotyped antigen receptor IGHV4-34/IGKV1. However, we only analyzed 3 
      patients. We conducted a retrospective study including lung tissue samples from 
      24 patients with pulmonary LCDD ((p)LCDD) matched with samples from 13 patients 
      with pulmonary kappa light chain amyloidosis ((p)AL amyloidosis) used as controls. 
      Mass spectrometry-based proteomics identified immunoglobulin kappa peptides as the 
      main protein component of the tissue deposits in all patients. Interestingly, in 
      (p)LCDD, IGKV1 was the most common kappa family detected (86.4%), and IGKV1-8 was 
      overrepresented compared with (p)AL amyloidosis (75% vs 11.1%, P = .0033). 
      Furthermore, IGKV1-8 was predominantly associated with a diffuse cystic pattern 
      (94%) in (p)LCDD. In conclusion, the high frequency of IGKV1-8 usage in cystic 
      (p)LCDD constitutes an additional feature arguing for a specific entity distinct 
      from the systemic form that preferentially uses IGKV4-1.
CI  - (c) 2019 by The American Society of Hematology.
FAU - Camus, Mylene
AU  - Camus M
AD  - Departement d'Anatomie Pathologique, Institut Universitaire du Cancer, Toulouse, 
      France.
AD  - Institut de Pharmacologie et de Biologie Structurale, Universite de Toulouse, 
      Centre National de la Recherche Scientifique, Universite Paul Sabatier, Toulouse, 
      France.
FAU - Hirschi, Sandrine
AU  - Hirschi S
AD  - Service de Pneumologie, Centre Hospitalier Universitaire (CHU) Strasbourg, 
      Strasbourg, France.
FAU - Prevot, Gregoire
AU  - Prevot G
AD  - Service de Pneumologie, CHU Toulouse, Toulouse, France.
FAU - Chenard, Marie-Pierre
AU  - Chenard MP
AD  - Departement d'Anatomie et Cytologie Patologiques, CHU Strasbourg, Strasbourg, 
      France.
FAU - Mal, Herve
AU  - Mal H
AD  - Service de Pneumologie, Hopital Bichat, Paris, France.
FAU - Stern, Marc
AU  - Stern M
AUID- ORCID: 0000-0001-5275-6959
AD  - Service de Pneumologie, Hopital Foch, Suresnes, France.
FAU - Reynaud-Gaubert, Martine
AU  - Reynaud-Gaubert M
AD  - Service de Pneumologie, CHU Marseille, Marseille, France.
FAU - Gilhodes, Julia
AU  - Gilhodes J
AD  - Service de Biostatistiques, Institut Claudius Regaud Institut Universitaire du 
      Cancer-O, Toulouse, France; and.
FAU - Burlet-Schiltz, Odile
AU  - Burlet-Schiltz O
AD  - Institut de Pharmacologie et de Biologie Structurale, Universite de Toulouse, 
      Centre National de la Recherche Scientifique, Universite Paul Sabatier, Toulouse, 
      France.
FAU - Brousset, Pierre
AU  - Brousset P
AD  - Departement d'Anatomie Pathologique, Institut Universitaire du Cancer, Toulouse, 
      France.
AD  - Labex Toucan, Toulouse, France.
FAU - Colombat, Magali
AU  - Colombat M
AD  - Departement d'Anatomie Pathologique, Institut Universitaire du Cancer, Toulouse, 
      France.
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20190409
PL  - United States
TA  - Blood
JT  - Blood
JID - 7603509
RN  - 0 (Immunoglobulin Light Chains)
SB  - IM
MH  - Adult
MH  - Female
MH  - Humans
MH  - Immunoglobulin Light Chains/*genetics
MH  - Lung Diseases/*genetics/pathology
MH  - Male
MH  - Middle Aged
MH  - Paraproteinemias/*genetics/pathology
MH  - Proteomics
MH  - Retrospective Studies
EDAT- 2019/04/11 06:00
MHDA- 2020/01/10 06:00
CRDT- 2019/04/11 06:00
PHST- 2019/01/22 00:00 [received]
PHST- 2019/03/19 00:00 [accepted]
PHST- 2019/04/11 06:00 [pubmed]
PHST- 2020/01/10 06:00 [medline]
PHST- 2019/04/11 06:00 [entrez]
AID - S0006-4971(20)42453-X [pii]
AID - 10.1182/blood.2019898577 [doi]
PST - ppublish
SO  - Blood. 2019 Jun 27;133(26):2741-2744. doi: 10.1182/blood.2019898577. Epub 2019 
      Apr 9.