Primary plasma cell leukemia in the era of novel agents for myeloma – a multicenter retrospective analysis of outcome. (May 2018)

• Record Type: Journal Article
• Authors: Ganzel, Chezi; Rouvio, Ory; Avivi, Irit; Magen, Hila; Jarchowsky, Osnat; Herzog, Katrin; Cohen, Yossi; Tadmor, Tamar; Horwitz, Netanel A.; Leiba, Merav; Nagler, Arnon; Cohen, Yael; Bulvik, Shlomo; Polliack, Aaron; Rowe, Jacob M.; Gatt, Moshe E.
• Is Part Of: Leukemia research. Volume 68(2018)
• Page Start: 9
• Page End: 14
• Publication Date: 2018-05
• Subjects: Primary plasma cell leukemia -- Multiple myeloma -- Novel agents -- Immunomodulatory drugs (IMiDs); Leukemia -- Periodicals; Leukemia -- Periodicals; Leucémie -- Périodiques; Leukemia; Periodicals; Electronic journals; Electronic journals; 616.9941905
• DOI: 10.1016/j.leukres.2018.02.010
• Languages: English
• ISSNs: 0145-2126

Abstract

Highlights: One-fifth of the patients died in the first 2 months after diagnosis. The overall survival of those who survived the first 3 months was 22.5 months. There was a survival advantage for those who received IMiD and for those who underwent hematopoietic cell transplantation. In multivariate analysis, low performance status and increased uric acid were also associated with shorter overall survival. Abstract: Primary plasma cell leukemia (PPCL) is a rare form of multiple myeloma with a dismal prognosis. This retrospective multi-center study examines the national experience of PPCL in the era of novel agents. During 2002–2016, thirty-nine patients with PPCL were identified in 11 Israeli centers. One-fifth of them died in the first 2 months after diagnosis. The overall survival (OS) of those who survived the first 3 months was 22.5 months. About 70% of patients received at least one type of immunomodulatory drug (IMiD) and similarly proteasome inhibitor (PI) during treatment. There was a survival advantage for those who received IMiD but not for those who received PI or other type of standard dose chemotherapy. In multivariate analysis, low performance status and increased uric acid were also associated with shorter OS. In conclusion, this study demonstrates favorable impact of treatment with IMiDs and hematopoietic cell transplantation on the survival of PPCL patients.