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

Carlos Fernández de Larrea; Robert Kyle; Laura Rosiñol; Bruno Paiva; Monika Engelhardt; Saad Usmani; Jo Caers; Wilson Gonsalves; Fredrik Schjesvold; Giampaolo Merlini; Suzanne Lentzch; Enrique Ocio; Laurent Garderet; Philippe Moreau; Pieter Sonneveld; Ashraf Badros; Gösta Gahrton; Hartmut Goldschmidt; Sascha Tuchman; Hermann Einsele; Brian Durie; Baldeep Wirk; Pellegrino Musto; Patrick Hayden; Martin Kaiser; Jesús San Miguel; Joan Bladé; S. Vincent Rajkumar; Maria Victoria Mateos

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^*, Robert Kyle, Laura Rosiñol, Bruno Paiva, Monika Engelhardt, Saad Usmani, Jo Caers, Wilson Gonsalves, Fredrik Schjesvold, Giampaolo Merlini, Suzanne Lentzch, Enrique Ocio, Laurent Garderet, Philippe Moreau, Pieter Sonneveld, Ashraf Badros, Gösta Gahrton, Hartmut Goldschmidt, Sascha Tuchman, Hermann EinseleBrian Durie, Baldeep Wirk, Pellegrino Musto, Patrick Hayden, Martin Kaiser, Jesús San Miguel, Joan Bladé, S. Vincent Rajkumar, Maria Victoria Mateos

^*Corresponding author for this work

Hematology

Research output: Contribution to journal › Article › Academic › peer-review

35 Citations (Scopus)

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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.

Original language	English
Article number	192
Journal	Blood Cancer Journal
Volume	11
Issue number	12
DOIs	https://doi.org/10.1038/s41408-021-00587-0
Publication status	Published - 2 Dec 2021

Bibliographical note

Funding Information:
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, Oncopep-tides, 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.

Funding Information:
This work has been supported in part by grants from the Instituto de Salud Carlos III, Spanish Ministry of Health (FIS PI19/00669), Fondo Europeo de Desarrollo Regional (FEDER) and 2017SGR00792 (AGAUR; Generalitat de Catalunya).

Publisher Copyright:
© 2021, The Author(s).

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Primary plasma cell leukemia, consensus definition by the International Myeloma Working Group according to peripheral blood plasma cell percentageFinal published version, 805 KBLicence: CC BY

Cite this

Fernández de Larrea, C., Kyle, R., Rosiñol, L., Paiva, B., Engelhardt, M., Usmani, S., Caers, J., Gonsalves, W., Schjesvold, F., Merlini, G., Lentzch, S., Ocio, E., Garderet, L., Moreau, P., Sonneveld, P., Badros, A., Gahrton, G., Goldschmidt, H., Tuchman, S., ... Mateos, M. V. (2021). Primary plasma cell leukemia: consensus definition by the International Myeloma Working Group according to peripheral blood plasma cell percentage. Blood Cancer Journal, 11(12), [192]. https://doi.org/10.1038/s41408-021-00587-0

@article{e5a8f6d65b004d78ac74553be5566333,

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

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.",

author = "{Fern{\'a}ndez de Larrea}, Carlos and Robert Kyle and Laura Rosi{\~n}ol and Bruno Paiva and Monika Engelhardt and Saad Usmani and Jo Caers and Wilson Gonsalves and Fredrik Schjesvold and Giampaolo Merlini and Suzanne Lentzch and Enrique Ocio and Laurent Garderet and Philippe Moreau and Pieter Sonneveld and Ashraf Badros and G{\"o}sta Gahrton and Hartmut Goldschmidt and Sascha Tuchman and Hermann Einsele and Brian Durie and Baldeep Wirk and Pellegrino Musto and Patrick Hayden and Martin Kaiser and Miguel, {Jes{\'u}s San} and Joan Blad{\'e} and Rajkumar, {S. Vincent} and Mateos, {Maria Victoria}",

note = "Funding Information: 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, Oncopep-tides, 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. Funding Information: This work has been supported in part by grants from the Instituto de Salud Carlos III, Spanish Ministry of Health (FIS PI19/00669), Fondo Europeo de Desarrollo Regional (FEDER) and 2017SGR00792 (AGAUR; Generalitat de Catalunya). Publisher Copyright: {\textcopyright} 2021, The Author(s).",

year = "2021",

month = dec,

day = "2",

doi = "10.1038/s41408-021-00587-0",

language = "English",

volume = "11",

journal = "Blood Cancer Journal",

issn = "2044-5385",

publisher = "Nature Publishing Group",

number = "12",

}

Fernández de Larrea, C, Kyle, R, Rosiñol, L, Paiva, B, Engelhardt, M, Usmani, S, Caers, J, Gonsalves, W, Schjesvold, F, Merlini, G, Lentzch, S, Ocio, E, Garderet, L, Moreau, P, Sonneveld, P, Badros, A, Gahrton, G, Goldschmidt, H, Tuchman, S, Einsele, H, Durie, B, Wirk, B, Musto, P, Hayden, P, Kaiser, M, Miguel, JS, Bladé, J, Rajkumar, SV & Mateos, MV 2021, 'Primary plasma cell leukemia: consensus definition by the International Myeloma Working Group according to peripheral blood plasma cell percentage', Blood Cancer Journal, vol. 11, no. 12, 192. https://doi.org/10.1038/s41408-021-00587-0

TY - JOUR

T1 - Primary plasma cell leukemia

T2 - consensus definition by the International Myeloma Working Group according to peripheral blood plasma cell percentage

AU - Fernández de Larrea, Carlos

AU - Kyle, Robert

AU - Rosiñol, Laura

AU - Paiva, Bruno

AU - Engelhardt, Monika

AU - Usmani, Saad

AU - Caers, Jo

AU - Gonsalves, Wilson

AU - Schjesvold, Fredrik

AU - Merlini, Giampaolo

AU - Lentzch, Suzanne

AU - Ocio, Enrique

AU - Garderet, Laurent

AU - Moreau, Philippe

AU - Sonneveld, Pieter

AU - Badros, Ashraf

AU - Gahrton, Gösta

AU - Goldschmidt, Hartmut

AU - Tuchman, Sascha

AU - Einsele, Hermann

AU - Durie, Brian

AU - Wirk, Baldeep

AU - Musto, Pellegrino

AU - Hayden, Patrick

AU - Kaiser, Martin

AU - Miguel, Jesús San

AU - Bladé, Joan

AU - Rajkumar, S. Vincent

AU - Mateos, Maria Victoria

N1 - Funding Information: 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, Oncopep-tides, 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. Funding Information: This work has been supported in part by grants from the Instituto de Salud Carlos III, Spanish Ministry of Health (FIS PI19/00669), Fondo Europeo de Desarrollo Regional (FEDER) and 2017SGR00792 (AGAUR; Generalitat de Catalunya). Publisher Copyright: © 2021, The Author(s).

PY - 2021/12/2

Y1 - 2021/12/2

N2 - 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.

AB - 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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U2 - 10.1038/s41408-021-00587-0

DO - 10.1038/s41408-021-00587-0

M3 - Article

AN - SCOPUS:85120883552

SN - 2044-5385

VL - 11

JO - Blood Cancer Journal

JF - Blood Cancer Journal

IS - 12

M1 - 192

ER -

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

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