Plasma cell leukemia: Consensus statement on diagnostic requirements, response criteria and treatment recommendations by the International Myeloma Working Group

C. Fernández De Larrea, R. A. Kyle, B. G M Durie, H. Ludwig, S. Usmani, D. H. Vesole, R. Hajek, J. F. San Miguel, O. Sezer, P. Sonneveld, Shaji K Kumar, A. Mahindra, R. Comenzo, A. Palumbo, A. Mazumber, K. C. Anderson, P. G. Richardson, A. Z. Badros, J. Caers, M. CavoX. Leleu, M. A. Dimopoulos, C. S. Chim, R. Schots, A. Noeul, D. Fantl, U. H. Mellqvist, O. Landgren, Asher A Chanan Khan, P. Moreau, Rafael Fonseca, G. Merlini, J. J. Lahuerta, J. Bladé, R. Z. Orlowski, J. J. Shah

Research output: Contribution to journalArticle

126 Citations (Scopus)

Abstract

Plasma cell leukemia (PCL) is a rare and aggressive variant of myeloma characterized by the presence of circulating plasma cells. It is classified as either primary PCL occurring at diagnosis or as secondary PCL in patients with relapsed/refractory myeloma. Primary PCL is a distinct clinic-pathological entity with different cytogenetic and molecular findings. The clinical course is aggressive with short remissions and survival duration. The diagnosis is based upon the percentage (≥20%) and absolute number (≥2 × 10 9/l) of plasma cells in the peripheral blood. It is proposed that the thresholds for diagnosis be re-examined and consensus recommendations are made for diagnosis, as well as, response and progression criteria. Induction therapy needs to begin promptly and have high clinical activity leading to rapid disease control in an effort to minimize the risk of early death. Intensive chemotherapy regimens and bortezomib-based regimens are recommended followed by high-dose therapy with autologous stem cell transplantation if feasible. Allogeneic transplantation can be considered in younger patients. Prospective multicenter studies are required to provide revised definitions and better understanding of the pathogenesis of PCL.

Original languageEnglish (US)
Pages (from-to)780-791
Number of pages12
JournalLeukemia
Volume27
Issue number4
DOIs
StatePublished - Apr 2013

Fingerprint

Plasma Cell Leukemia
Plasma Cells
Therapeutics
Homologous Transplantation
Stem Cell Transplantation
Cytogenetics
Multicenter Studies
Prospective Studies
Drug Therapy
Survival

Keywords

  • bortezomib
  • cytogenetics
  • myeloma
  • plasma cell leukemia
  • prognosis
  • transplantation

ASJC Scopus subject areas

  • Hematology
  • Cancer Research
  • Anesthesiology and Pain Medicine

Cite this

Fernández De Larrea, C., Kyle, R. A., Durie, B. G. M., Ludwig, H., Usmani, S., Vesole, D. H., ... Shah, J. J. (2013). Plasma cell leukemia: Consensus statement on diagnostic requirements, response criteria and treatment recommendations by the International Myeloma Working Group. Leukemia, 27(4), 780-791. https://doi.org/10.1038/leu.2012.336

Plasma cell leukemia : Consensus statement on diagnostic requirements, response criteria and treatment recommendations by the International Myeloma Working Group. / Fernández De Larrea, C.; Kyle, R. A.; Durie, B. G M; Ludwig, H.; Usmani, S.; Vesole, D. H.; Hajek, R.; San Miguel, J. F.; Sezer, O.; Sonneveld, P.; Kumar, Shaji K; Mahindra, A.; Comenzo, R.; Palumbo, A.; Mazumber, A.; Anderson, K. C.; Richardson, P. G.; Badros, A. Z.; Caers, J.; Cavo, M.; Leleu, X.; Dimopoulos, M. A.; Chim, C. S.; Schots, R.; Noeul, A.; Fantl, D.; Mellqvist, U. H.; Landgren, O.; Chanan Khan, Asher A; Moreau, P.; Fonseca, Rafael; Merlini, G.; Lahuerta, J. J.; Bladé, J.; Orlowski, R. Z.; Shah, J. J.

In: Leukemia, Vol. 27, No. 4, 04.2013, p. 780-791.

Research output: Contribution to journalArticle

Fernández De Larrea, C, Kyle, RA, Durie, BGM, Ludwig, H, Usmani, S, Vesole, DH, Hajek, R, San Miguel, JF, Sezer, O, Sonneveld, P, Kumar, SK, Mahindra, A, Comenzo, R, Palumbo, A, Mazumber, A, Anderson, KC, Richardson, PG, Badros, AZ, Caers, J, Cavo, M, Leleu, X, Dimopoulos, MA, Chim, CS, Schots, R, Noeul, A, Fantl, D, Mellqvist, UH, Landgren, O, Chanan Khan, AA, Moreau, P, Fonseca, R, Merlini, G, Lahuerta, JJ, Bladé, J, Orlowski, RZ & Shah, JJ 2013, 'Plasma cell leukemia: Consensus statement on diagnostic requirements, response criteria and treatment recommendations by the International Myeloma Working Group', Leukemia, vol. 27, no. 4, pp. 780-791. https://doi.org/10.1038/leu.2012.336
Fernández De Larrea, C. ; Kyle, R. A. ; Durie, B. G M ; Ludwig, H. ; Usmani, S. ; Vesole, D. H. ; Hajek, R. ; San Miguel, J. F. ; Sezer, O. ; Sonneveld, P. ; Kumar, Shaji K ; Mahindra, A. ; Comenzo, R. ; Palumbo, A. ; Mazumber, A. ; Anderson, K. C. ; Richardson, P. G. ; Badros, A. Z. ; Caers, J. ; Cavo, M. ; Leleu, X. ; Dimopoulos, M. A. ; Chim, C. S. ; Schots, R. ; Noeul, A. ; Fantl, D. ; Mellqvist, U. H. ; Landgren, O. ; Chanan Khan, Asher A ; Moreau, P. ; Fonseca, Rafael ; Merlini, G. ; Lahuerta, J. J. ; Bladé, J. ; Orlowski, R. Z. ; Shah, J. J. / Plasma cell leukemia : Consensus statement on diagnostic requirements, response criteria and treatment recommendations by the International Myeloma Working Group. In: Leukemia. 2013 ; Vol. 27, No. 4. pp. 780-791.
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AU - Ludwig, H.

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AU - Vesole, D. H.

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AU - San Miguel, J. F.

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AU - Mahindra, A.

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AU - Badros, A. Z.

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AU - Moreau, P.

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AU - Merlini, G.

AU - Lahuerta, J. J.

AU - Bladé, J.

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AU - Shah, J. J.

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