Fiche publication
Date publication
avril 2019
Journal
Blood
Auteurs
Membres identifiés du Cancéropôle Est :
Pr CHENARD Marie-Pierre
Tous les auteurs :
Camus M, Hirschi S, Prevot G, Chenard MP, Mal H, Stern M, Reynaud-Gaubert M, Gilhodes J, Burlet-Schiltz O, Brousset P, Colombat M
Lien Pubmed
Résumé
We previously reported a new form of LCDD presenting as diffuse cystic lung disorder that differs from the usual systemic form, with respect to the 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 analyzed only 3 patients. Herein, we conducted a retrospective study including lung tissue samples from 24 patients with pulmonary LCDD (LCDD) matched with samples from 13 patients with pulmonary AL kappa 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 LCDD, IGKV1 was the most common kappa family detected (86.4%) and, IGKV1-8 was overrepresented compared with pulmonary AL kappa amyloidosis (75% vs 11.1%, p=0.0033). Furthermore, IGKV1-8 was predominantly associated with a diffuse cystic pattern (94%) in LCDD. In conclusion, high frequency of IGKV1-8 usage in cystic LCDD constitutes an additional feature arguing for a specific entity distinct from the systemic form that uses preferentially IGKV4-1.
Référence
Blood. 2019 Apr 9;:
