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. 2021 Dec 2;11(12):192.
doi: 10.1038/s41408-021-00587-0.

Primary plasma cell leukemia: consensus definition by the International Myeloma Working Group according to peripheral blood plasma cell percentage

Carlos Fernández de Larrea  1 , Robert Kyle  2 , Laura Rosiñol  3 , Bruno Paiva  4 , Monika Engelhardt  5 , Saad Usmani  6 , Jo Caers  7 , Wilson Gonsalves  2 , Fredrik Schjesvold  8 , Giampaolo Merlini  9 , Suzanne Lentzch  10 , Enrique Ocio  11 , Laurent Garderet  12 , Philippe Moreau  13 , Pieter Sonneveld  14 , Ashraf Badros  15 , Gösta Gahrton  16 , Hartmut Goldschmidt  17 , Sascha Tuchman  18 , Hermann Einsele  19 , Brian Durie  20 , Baldeep Wirk  21 , Pellegrino Musto  22 , Patrick Hayden  23 , Martin Kaiser  24 , Jesús San Miguel  4 , Joan Bladé  3 , S Vincent Rajkumar  2 , Maria Victoria Mateos  11
Affiliations

Primary plasma cell leukemia: consensus definition by the International Myeloma Working Group according to peripheral blood plasma cell percentage

Carlos Fernández de Larrea et al. Blood Cancer J. .

Abstract

Primary plasma cell leukemia (PCL) has a consistently ominous prognosis, even after progress in the last decades. PCL deserves a prompt identification to start the most effective treatment for this ultra-high-risk disease. The aim of this position paper is to revisit the diagnosis of PCL according to the presence of circulating plasma cells in patients otherwise meeting diagnostic criteria of multiple myeloma. We could identify two retrospective series where the question about what number of circulating plasma cells in peripheral blood should be used for defining PCL. The presence of ≥5% circulating plasma cells in patients with MM had a similar adverse prognostic impact as the previously defined PCL. Therefore, PCL should be defined by the presence of 5% or more circulating plasma cells in peripheral blood smears in patients otherwise diagnosed with symptomatic multiple myeloma.

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Conflict of interest statement

BP reports honoraria for lectures from and membership on advisory boards with Adaptive, Amgen, Bristol-Myers Squibb-Celgene, Creative BioLabs, Gilead, Janssen, Sanofi and Takeda; unrestricted grants from Celgene, EngMab, Roche, Sanofi, and Takeda; and consultancy for Bristol-Myers Squibb-Celgene, Janssen, Sanofi, and Takeda. LR reports honoraria from Janssen, BMS-Celgene, Amgen, Takeda, Sanofi, GSK. SZU reports grants and personal fees from Amgen, personal fees from Abbvie, grants from BMS, grants and personal fees from Celgene, personal fees from MundiPharma, grants from Pharmacyclics, grants and personal fees from Sanofi, grants and personal fees from Seattle Genetics, grants and personal fees from Janssen, grants and personal fees from Takeda, grants and personal fees from SkylineDX, grants and personal fees from Merck, grants and personal fees from GSK, outside the submitted work. FS reports honoraria from Abbvie, Amgen, BMS, Janssen, Novartis, Oncopeptides, Sanofi, Schain, SkyliteDX, Takeda; consultancy or advisory board from Celgene, Janssen, Oncopeptides, Sanofi, and unrestricted grants from Celgene, GSK, Janssen, Oncopeptides, Sanofi. SL reports honoraria or consultancy from Sorrento, Janssen, Celularity, Abbvie, GSK, Takeda, Karyopharm, Sanofi, Oncopetide and Caelum Biosciences; research grants from Karyopharm, Sanofi; and holds shares and serves on board of directors for Caelum Biosciences. EMO reports honoraria or consultancy funding from Janssen, BMS, Sanofi, GSK, Oncopeptides, Takeda, Pfizer and Amgen. LG reports consultancy or advisory boards from Takeda, Amgen, BMS/Celgene, and Janssen. GG reports honoraria or consultancy from Fujimoto Pharmaceutical Corporation, Japan. HG reports grants and/or provision of Investigational Medicinal Product from Amgen, BMS, Celgene, Chugai, Dietmar-Hopp-Foundation, Janssen, Johns Hopkins University, Sanofi; research grants from Amgen, BMS, Celgene, Chugai, Janssen, Incyte, Molecular Partners, Merck Sharp and Dohme (MSD), Sanofi, Mundipharma GmbH, Takeda, Novartis; and consultancy or advisory boards from Adaptive Biotechnology, Amgen, BMS, Celgene, Janssen, Sanofi, Takeda; and honoraria from Amgen, BMS, Celgene, Chugai, GlaxoSmithKline (GSK), Janssen, Novartis, Sanofi. SAT reports grants received from Karyopharm, Sanofi Genzyme and Caelum; and honoraria from Karyopharm, Caelum, Sanofi Genzyme, Oncopeptides, and Shattuck Labs. HE reports honoria or consultancy and unrestricted grants from Janssen, BMS/Celgene, Amgen, Novartis, Takeda, Sanofi, GSK. BGMD reports consultancy or advisory boards from Takeda, Amgen, BMS/Celgene, and Janssen. MK reports consultancy from AbbVie, Amgen, BMS/Celgene, GSK, Janssen, Karyopharm, Seattle Genetics, Takeda; research funding from BMS/Celgene, Janssen; and educational or travel support from BMS/Celgene, Janssen, and Takeda. JSM reports consultancy or advisory boards from AbbVie, Amgen, BMS, Celgene, GSK, Janssen, Karyopharm, MSD, Novartis, Roche, Sanofi, Takeda, SecuraBio, and Regeneron. MVM reports honoraria or consultancy from Janssen, BMS-Celgene, Takeda, Amgen, Abbvie, GSK, Sanofi, Oncopeptides, Pfizer, Regeneron, Roche, Sea-Gen, Bluebird-bio. The remaining authors declare no competing interests.

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