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Cytogenetic and therapeutic characterization of primary plasma cell leukemia: the IFM experience

Leukemia volume 26, pages 158–159 (2012)Cite this article

During the past decade, tremendous improvements have been observed in the outcome of patients with multiple myeloma. This significant improvement is mainly related to the availability of novel drugs, such as thalidomide, bortezomib and lenalidomide. Using a combination of these novel drugs, combined with high-dose melphalan, the median overall survival of patients under 65 years of age is currently around 8–10 years. However, despite these marked improvements, some patients do still present a very poor outcome, with fatal evolution within a few months. Several prognostic parameters have been proposed to identify these ultra high-risk patients. One of the most important factors is described by the ISS stage, especially patients with stage 3, that is, with a β2-microglobulin level >5.5 mg/l.1 The second very important factor is related to chromosomal abnormalities, especially t(4;14) and del(17p).2 Apart from these poor prognosis factors, another strong parameter is a leukemic presentation at diagnosis. So far, very few large studies on this rare form of myeloma (1–2% of the cases at diagnosis) have been reported.3, 4, 5 Our objective was to describe the prognostic parameters of such patients, and to look at their outcome. Furthermore, a secondary objective was to analyze this outcome in the context of the novel drug (thalidomide, bortezomib and lenalidomide) era.

Plasma cell leukemia (PCL) is arbitrarily defined as the presence of 20% of circulating plasma cells (PCs), or an absolute number of PCs >2 G/l. Two types of PCL have been described: primitive (at diagnosis, or pPCL) and secondary (at relapse, or sPCL). The physiopathology of PCL is largely unknown. It is thought that phenotypic changes in surface antigen expression allow the PCs to quit the bone marrow niche and to disseminate in the blood circulation. Cytogenetic characterization of primary PCL has been previously reported, with a higher incidence of t(11;14) and t(14;16).6 Very few reports have analyzed the outcome of patients with pPCL. Most of them include only a small number of patients, but all converge towards a dismal outcome.

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Authors and Affiliations

  1. Hematology Laboratory, University Hospital,

    H Avet-Loiseau

  2. INSERM U892, Nantes, France

    H Avet-Loiseau

  3. Hematology Department, University Hospital, Toulouse, France

    M Roussel

  4. Statistics Department, Centre René Gauducheau, Nantes, France

    L Campion

  5. Hematology Department, University Hospital, Lille, France

    X Leleu

  6. Hematology Department, University Hospital, Bordeaux, France

    G Marit

  7. Hematology Department, Department Hospital, Vannes, France

    H Jardel

  8. Hematology Department, University Hospital, Angers, France

    M Dib

  9. Internal Medicine Department, University Hospital, Rennes, France

    O Decaux

  10. Hematology Department, University Hospital, Rennes, France

    T Lamy

  11. Hematology Department, Department Hospital, La Roche/Yon, France

    M Tiab

  12. Hematology Department, Department Hospital, Annecy, France

    P Cony-Makhoul

  13. Hematology Department, Department Hospital, Bayonne, France

    A Banos

  14. Hematology Department, Department Hospital, Avignon, France

    G Lepeu

  15. Internal Medicine Department, Department Hospital, Dax, France

    F Lifermann

  16. Hematology Department, University Hospital, Caen, France

    M Macro

  17. Hematology Department, University Hospital, Reims, France

    B Kolb

  18. Hematology Department, University Hospital, Nancy, France

    C Hulin

  19. Hematology Department, University Hospital, Dijon, France

    D Caillot

  20. Hematology Department, University Hospital, Lyon, France

    C Traulle

  21. Hematology Laboratory, Institut Curie, Paris, France

    C Mathiot

  22. Hematology Department, University Hospital, Amiens, France

    B Royer

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  1. H Avet-Loiseau
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  2. M Roussel
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  8. O Decaux
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  12. A Banos
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  13. G Lepeu
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  14. F Lifermann
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  15. M Macro
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  17. C Hulin
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  18. D Caillot
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  19. C Traulle
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Correspondence to H Avet-Loiseau.

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Avet-Loiseau, H., Roussel, M., Campion, L. et al. Cytogenetic and therapeutic characterization of primary plasma cell leukemia: the IFM experience. Leukemia 26, 158–159 (2012). https://doi.org/10.1038/leu.2011.176

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