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Review
. 2013 Apr;27(4):780-91.
doi: 10.1038/leu.2012.336. Epub 2012 Nov 21.

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

C Fernández de Larrea  1 , R A Kyle, B G M Durie, H Ludwig, S Usmani, D H Vesole, R Hajek, J F San Miguel, O Sezer, P Sonneveld, S K Kumar, A Mahindra, R Comenzo, A Palumbo, A Mazumber, K C Anderson, P G Richardson, A Z Badros, J Caers, M Cavo, X LeLeu, M A Dimopoulos, C S Chim, R Schots, A Noeul, D Fantl, U-H Mellqvist, O Landgren, A Chanan-Khan, P Moreau, R Fonseca, G Merlini, J J Lahuerta, J Bladé, R Z Orlowski, J J Shah; International Myeloma Working Group
Collaborators, Affiliations
Review

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

C Fernández de Larrea et al. Leukemia. 2013 Apr.

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.

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Figures

Figure 1
Figure 1
Abdominal tomography showing a focal lesion (40 mm), highlighted by the arrow, suggestive of metastasic infiltration of the liver in a patients with plasma cell leukemia.
Figure 2
Figure 2
PET/CT-scan of a patient with primary PCL showing focal bone lesion with increased uptake of FDG in vertebrae, ribs and pelvis.
Figure 3
Figure 3
Conventional morphology in plasma cell leukemia cases shows bone marrow infiltration (Panel A), with circulating plasma cells (Panel B) and frequent extramedullary involvement, as hepatic infiltration (Panel C).
Figure 3
Figure 3
Conventional morphology in plasma cell leukemia cases shows bone marrow infiltration (Panel A), with circulating plasma cells (Panel B) and frequent extramedullary involvement, as hepatic infiltration (Panel C).
Figure 4
Figure 4
Treatment algorithm for primary plasma cell leukemia
Figure 5
Figure 5
Treatment algorithm for secondary plasma cell leukemia or relapsed primary plasma cell leukemia
See this image and copyright information in PMC

References

    1. Gluzinski A, Reichentein M. Myeloma und leucaemia lymphatica plasmocellularis. Wien Klin Wochenschr. 1906;19:336.
    1. Kyle RA, Maldonado JE, Bayrd ED. Plasma cell leukemia. Report on 17 cases. Arch Intern Med. 1974;133:813–818. - PubMed
    1. Noel P, Kyle RA. Plasma cell leukemia: an evaluation of response to therapy. Am J Med. 1987;83:1062–1068. - PubMed
    1. Dimopoulos MA, Palumbo A, Delasalle KB, Alexanian R. Primary plasma cell leukaemia. Br J Haematol. 1994;88:754–759. - PubMed
    1. García-Sanz R, Orfao A, González M, Tabernero MD, Bladé J, Moro MJ, et al. Primary plasma cell leukemia: clinical, immunophenotypic, DNA ploidy, and cytogenetic characteristics. Blood. 1999;93:1032–1037. - PubMed

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