Primary Plasma Cell Leukemia: Consensus Definition by the International Myeloma Working Group According to Peripheral Blood Plasma Cell Percentage

Overview

Journal Blood Cancer J

Date 2021 Dec 3

PMID 34857730

Citations 51

Authors

Carlos Fernandez de Larrea

Robert Kyle

Laura Rosinol

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

Gosta Gahrton

Hartmut Goldschmidt

Sascha Tuchman

Hermann Einsele

Brian Durie

Baldeep Wirk

Pellegrino Musto

Patrick Hayden

Martin Kaiser

Jesus San Miguel

Joan Blade

S Vincent Rajkumar

Maria Victoria Mateos

Affiliations

Soon will be listed here.

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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References

Royer B, Minvielle S, Diouf M, Roussel M, Karlin L, Hulin C . Bortezomib, Doxorubicin, Cyclophosphamide, Dexamethasone Induction Followed by Stem Cell Transplantation for Primary Plasma Cell Leukemia: A Prospective Phase II Study of the Intergroupe Francophone du Myélome. J Clin Oncol. 2016; 34(18):2125-32. DOI: 10.1200/JCO.2015.63.1929. View

Blade J, Kyle R . Nonsecretory myeloma, immunoglobulin D myeloma, and plasma cell leukemia. Hematol Oncol Clin North Am. 2000; 13(6):1259-72. DOI: 10.1016/s0889-8588(05)70125-8. View

Katodritou E, Terpos E, Kelaidi C, Kotsopoulou M, Delimpasi S, Kyrtsonis M . Treatment with bortezomib-based regimens improves overall response and predicts for survival in patients with primary or secondary plasma cell leukemia: Analysis of the Greek myeloma study group. Am J Hematol. 2013; 89(2):145-50. DOI: 10.1002/ajh.23600. View

Jurczyszyn A, Radocha J, Davila J, Fiala M, Gozzetti A, Grzasko N . Prognostic indicators in primary plasma cell leukaemia: a multicentre retrospective study of 117 patients. Br J Haematol. 2018; 180(6):831-839. DOI: 10.1111/bjh.15092. View

Vagnoni D, Travaglini F, Pezzoni V, Ruggieri M, Bigazzi C, Dalsass A . Circulating plasma cells in newly diagnosed symptomatic multiple myeloma as a possible prognostic marker for patients with standard-risk cytogenetics. Br J Haematol. 2015; 170(4):523-31. DOI: 10.1111/bjh.13484. View

Usmani S, Nair B, Qu P, Hansen E, Zhang Q, Petty N . Primary plasma cell leukemia: clinical and laboratory presentation, gene-expression profiling and clinical outcome with Total Therapy protocols. Leukemia. 2012; 26(11):2398-405. PMC: 3426639. DOI: 10.1038/leu.2012.107. View

Lionetti M, Musto P, Di Martino M, Fabris S, Agnelli L, Todoerti K . Biological and clinical relevance of miRNA expression signatures in primary plasma cell leukemia. Clin Cancer Res. 2013; 19(12):3130-42. DOI: 10.1158/1078-0432.CCR-12-2043. View

Kyle R, Maldonado J, BAYRD E . Plasma cell leukemia. Report on 17 cases. Arch Intern Med. 1974; 133(5):813-8. DOI: 10.1001/archinte.133.5.813. View

Billadeau D, Van Ness B, Kimlinger T, Kyle R, Therneau T, Greipp P . Clonal circulating cells are common in plasma cell proliferative disorders: a comparison of monoclonal gammopathy of undetermined significance, smoldering multiple myeloma, and active myeloma. Blood. 1996; 88(1):289-96. View

10.

Granell M, Calvo X, Garcia-Guinon A, Escoda L, Abella E, Martinez C . Prognostic impact of circulating plasma cells in patients with multiple myeloma: implications for plasma cell leukemia definition. Haematologica. 2017; 102(6):1099-1104. PMC: 5451342. DOI: 10.3324/haematol.2016.158303. View

11.

Garcia-Sanz R, Orfao A, Gonzalez M, Tabernero M, Blade J, Moro M . Primary plasma cell leukemia: clinical, immunophenotypic, DNA ploidy, and cytogenetic characteristics. Blood. 1999; 93(3):1032-7. View

12.

Drake M, Iacobelli S, van Biezen A, Morris C, Apperley J, Niederwieser D . Primary plasma cell leukemia and autologous stem cell transplantation. Haematologica. 2010; 95(5):804-9. PMC: 2864387. DOI: 10.3324/haematol.2009.013334. View

13.

Todoerti K, Calice G, Trino S, Simeon V, Lionetti M, Manzoni M . Global methylation patterns in primary plasma cell leukemia. Leuk Res. 2018; 73:95-102. DOI: 10.1016/j.leukres.2018.09.007. View

14.

Musto P, Simeon V, Martorelli M, Petrucci M, Cascavilla N, Di Raimondo F . Lenalidomide and low-dose dexamethasone for newly diagnosed primary plasma cell leukemia. Leukemia. 2013; 28(1):222-5. DOI: 10.1038/leu.2013.241. View

15.

Tiedemann R, Gonzalez-Paz N, Kyle R, Santana-Davila R, Price-Troska T, Van Wier S . Genetic aberrations and survival in plasma cell leukemia. Leukemia. 2008; 22(5):1044-52. PMC: 3893817. DOI: 10.1038/leu.2008.4. View

16.

An G, Qin X, Acharya C, Xu Y, Deng S, Shi L . Multiple myeloma patients with low proportion of circulating plasma cells had similar survival with primary plasma cell leukemia patients. Ann Hematol. 2014; 94(2):257-64. DOI: 10.1007/s00277-014-2211-0. View

17.

Sanoja-Flores L, Flores-Montero J, Garces J, Paiva B, Puig N, Garcia-Mateo A . Next generation flow for minimally-invasive blood characterization of MGUS and multiple myeloma at diagnosis based on circulating tumor plasma cells (CTPC). Blood Cancer J. 2018; 8(12):117. PMC: 6242818. DOI: 10.1038/s41408-018-0153-9. View

18.

Nowakowski G, Witzig T, Dingli D, Tracz M, Gertz M, Lacy M . Circulating plasma cells detected by flow cytometry as a predictor of survival in 302 patients with newly diagnosed multiple myeloma. Blood. 2005; 106(7):2276-9. PMC: 1895270. DOI: 10.1182/blood-2005-05-1858. View

19.

Gonsalves W, Rajkumar S, Gupta V, Morice W, Timm M, Singh P . Quantification of clonal circulating plasma cells in newly diagnosed multiple myeloma: implications for redefining high-risk myeloma. Leukemia. 2014; 28(10):2060-5. PMC: 4162866. DOI: 10.1038/leu.2014.98. View

20.

. Criteria for the classification of monoclonal gammopathies, multiple myeloma and related disorders: a report of the International Myeloma Working Group. Br J Haematol. 2003; 121(5):749-57. View