Fiche publication
Date publication
février 2022
Journal
Blood
Auteurs
Membres identifiés du Cancéropôle Est :
Dr CHRETIEN Marie-Lorraine
Tous les auteurs :
Cazaubiel T, Leleu X, Perrot A, Manier S, Buisson L, Mahéo S, Do Souto Ferreira L, Lannes R, Pavageau L, Hulin C, Marolleau JP, Voillat L, Belhadj K, Divoux M, Slama B, Brechignac S, Macro M, Stoppa AM, Laurence S, Orsini Piocelle F, Fontan J, Chretien ML, DeMarquette H, Mohty M, Schavgoulidze A, Avet-Loiseau H, Corre J
Lien Pubmed
Résumé
Primary plasma cell leukemia (pPCL) is an aggressive form of multiple myeloma (MM) that has not benefited from recent therapeutic advances in the field. Because very rare and heterogeneous, it remains poorly understood at the molecular level. To address this issue, we performed DNA and RNA sequencing of sorted plasma cells from a large cohort of 90 newly diagnosed pPCL, and compared to MM. We observed that pPCL presents a specific genomic landscape with a high prevalence of t(11;14) (about half) and high-risk genomic features such as del(17p), gain 1q, del(1p32). In addition, pPCL displays a specific transcriptome when compared to MM. We then aimed at specifically characterize pPCL with t(11;14). We observed that this sub-entity displayed significantly fewer adverse cytogenetic abnormalities. This translated into better overall survival when compared to pPCL without t(11;14) (39.2 months vs 17.9 months, p=0.002). Finally, pPCL with t(11;14) displayed a specific transcriptome, including differential expression of BCL2 family members. This study is the largest series of patients with pPCL reported so far.
Référence
Blood. 2022 Feb 16;:
