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Non-nodal type of mantle cell lymphoma is a specific biological and clinical subgroup of the disease

Leukemia volume 26, pages 1895–1898 (2012)Cite this article

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Mantle cell lymphoma (MCL) is an aggressive B-cell neoplasm with a median survival of the patients of 3–5 years.1 This aggressive behavior has been related to its genetic and molecular pathogenesis that integrates the deregulation of cell proliferation due to the t(11;14)(q13;q32) and cyclin D1 overexpression, and the accumulation of a high number of chromosomal aberrations mainly targeting genes related to DNA damage response and the cell survival pathways.2 Thus, it is usually recommended that patients with MCL should be treated with chemotherapeutic regimens immediately following their diagnosis. However, this attitude is being reconsidered, due in part to the increasing recognition of subsets of patients that do not need therapy for a long period of time3, 4, 5, 6, 7 and the observation that deferral of treatment does not seem to impair their global outcome.3, 5 The identification of asymptomatic patients at diagnosis that may benefit from initial watch and wait approach is challenging because some studies have shown that intensive treatment may improve the survival of patients with MCL.8 Therefore, it is of paramount importance to develop clinical and biological criteria that may assist in the selection of the optimal individual management for patients with MCL.

In a recent study, we compared a subgroup of MCL patients with a very indolent clinical behavior that did not received chemotherapy for more than 2 years with a subset of patients that required treatment at diagnosis.4 The indolent MCL had a profile of clinical and biological features that differed from the conventional MCL (cMCL), suggesting that at least a subset of cases might correspond to a particular biological subtype of the disease. One of the major findings was the differential expression of a small signature of 13 genes, including SOX11, that was highly expressed in cMCL but negative or very low in indolent tumors.4

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Acknowledgements

This study has been supported by ‘Instituto de Salud Carlos III, Fondo Investigaciones Sanitarias’ (FIS06/0150 and PI08/0077) (SB), the Spanish Ministry of Science and Innovation SAF 2008-03630 (EC), ‘Red Temática de Investigación Cooperativa de Cáncer’ (RTICC) RD06/0020/0039 (EC), RD06/0020/0051 (ALG) and RD06/0020/0014 (DC), Instituto de Salud Carlos III ‘Beca Predoctoral de Formación en Investigación en Salud’ (FI08/00437) (CR), Ministry of Science and Innovation ‘Formación de Personal Investigador’ (BES-2007-16330) (AN), European Regional Development Fund (ERDF) ‘Una manera de fer Europa’, European Mantle Cell Lymphoma Network, and Generalitat de Catalunya (2009-SGR-992) (EC).

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Author notes

  1. C Royo and E Campo: These authors share authorship.

  2. A Navarro and S Beà: These authors share senior authorship.

Authors and Affiliations

  1. Department of Pathology, Hematopathology Unit, Hospital Clinic, University of Barcelona, IDIBAPS, Barcelona, Spain

    C Royo, A Navarro, G Clot, I Salaverria, P Jares, D Colomer, M C Vegliante, V Fernandez, E Campo & S Beà

  2. Institute of Human Genetics, University Hospital Schleswig-Holstein Campus Kiel/Christian-Albrechts University Kiel, Kiel, Germany

    I Salaverria & R Siebert

  3. Department of Hematology, Hospital Clinic, Barcelona, Spain

    E Giné & A López-Guillermo

  4. Hematology Branch, National Institute of Health, Bethesda, MD, USA

    A Wiestner & W H Wilson

  5. Insitute of Pathology, University of Würzburg, Würzburg, Germany

    E M Hartmann & A Rosenwald

  6. Addenbrooke’s Hospital, Cambridge, UK

    N Trim

  7. School of Pathology and Laboratory Medicine, The University of Western Australia, Nedlands, Western Australia, Australia

    W N Erber

  8. University of Pittsburgh School of Medicine, Pittsburgh, PA, USA

    S H Swerdlow

  9. Department of Pathology, University Hospital Schleswig-Holstein Campus Kiel/Christian-Albrechts University Kiel, Kiel, Germany

    W Klapper

  10. MRC Toxicology Unit, Leicester University, Leicester, UK

    M J S Dyer

  11. Hospital de Jarrio, Asturias, Spain

    M Vargas-Pabón

  12. Department of Pathology, Robert-Bosch-Krankenhaus and Dr Margarete Fischer Institute of Clinical Pharmacology, Stuttgart, Germany

    G Ott

Authors
  1. C Royo
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Corresponding author

Correspondence to S Beà.

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Royo, C., Navarro, A., Clot, G. et al. Non-nodal type of mantle cell lymphoma is a specific biological and clinical subgroup of the disease. Leukemia 26, 1895–1898 (2012). https://doi.org/10.1038/leu.2012.72

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