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Lymphoma

Blastic variant of mantle cell lymphoma: a rare but highly aggressive subtype

Leukemia volume 15, pages 1785–1791 (2001)Cite this article

Abstract

The blastic variant (BV) form of mantle cell lymphoma (MCL) is considered to be a very aggressive subtype of non-Hodgkin's lymphoma (NHL). In order to determine its clinico-biological features and response to therapy we studied 33 patients (17%) out of 187 suffering from MCL who were diagnosed with a BV of MCL. Blastic variant was diagnosed according to histopathological patterns, immunophenotyping, and bcl1 gene rearrangement and/or cyclin D1 overexpression. Three patients initially diagnosed with large cell NHL were classified as BV. Patients received front-line therapy including CHOP-like regimen or CVP (n = 29), or chlorambucil (n = 4) and CHOP or ESAP as second-line therapy. High-dose intensification with stem cell transplantation (SCT) was performed in 11 cases (autoSCT, n = 8; alloSCT, n = 3). All but two patients were in complete remission (CR) at the time of transplant (CR1, n = 5; CR2, n = 4). Clinical and biological characteristics did not differ from those of the common form of MCL. The median age was 62 years (29–80), with a sex ratio (M/F) of 2.6:1. Of the 33 patients, 66% had extranodal site involvement, 85% had an Ann Arbor stage IV, and 82% had peripheral lymphadenopathy. Circulating lymphomatous cells were seen in 48% of cases. Twelve patients (36%) entered a CR1 with a median duration of 11 months. Fifteen patients (46%) failed to respond and rapidly died of progressive disease. Second-line therapy led to a 26% (6/23) CR2 rate. Nine patients relapsed after high-dose therapy. Twenty-two of the 33 patients (66%) died of refractory or progressive disease. Median overall survival (OS) time was 14.5 months for the 33 BV patients as compared to 53 months for the 154 patients with a common form of MCL, P <0.0001. In the univariate analysis, OS was influenced by age, extranodal site involvement, circulating lymphomatous cells, and international prognosis index (IPI). In the multivariate analysis, only IPI affected OS: patients with IPI ⩾2 had 8 months median OS as compared to 36 months median OS for patients with IPI <2, P = 0.003. Blastic variant is one of the worst forms of NHL. An improved recognition of BV of MCL is required, particularly in high-grade CD5+ NHL using immunophenotyping and bcl1 molecular study. Standard therapy using anthracycline or even high-dose intensification produce poor results and an alternative treatment should be proposed to such patients.

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Acknowledgements

This work was supported by ADHO-Rennes (Association Développement Hématologie Oncologie).

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Authors and Affiliations

  1. On behalf of the French GOELAMS Group, France

    M Bernard, R Gressin, B Drénou, S Caulet-Maugendre, P Tass, N Milpied, L Voilat, A Sadoun, C Ghandour, M Hunault, R Leloup, L Mannone & T Lamy

  2. Service d'Hématologie, CHU de Grenoble, France

    R Gressin

  3. Service d'Hématologie Clinique, Hôpital Necker, Paris, France

    F Lefrère & O Hermine

  4. Laboratoire d'Hématologie, CHU de Rennes, France

    B Drénou

  5. Service d'Epidémiologie, CHU de Rennes, France

    B Branger

  6. Laboratoire d'Anatomie Pathologie, CHU de Rennes, France

    S Caulet-Maugendre

  7. Laboratoire d'Anatomie Pathologie, CHU de Rennes, France

    P Tass

  8. Laboratoire d'Anatomo-pathologie, Hôpital Necker, Paris, France

    N Brousse

  9. Laboratoire d'Hématologie Biologique, Hôpital Necker, Paris, France

    F Valensi

  10. Service d'Hématologie, CHU de Nantes, France

    N Milpied

  11. Service d'Hématologie, CHU de Besançon, France

    L Voilat

  12. Service d'Hématologie, CHU de Poitiers, France

    A Sadoun

  13. Clinique d'Hématologie, Cesson-Sévigné, France

    C Ghandour

  14. Service d'Hématologie, CHU d'Angers, France

    M Hunault

  15. Service d'Hématologie, CHU d'Orléans, France

    R Leloup

  16. Service d'Hématologie, CHU de Dijon, France

    L Mannone

  17. Service d'Hématologie, Hôpital Pontchaillon, CHU de Rennes, Rennes, France

    T Lamy

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Bernard, M., Gressin, R., Lefrère, F. et al. Blastic variant of mantle cell lymphoma: a rare but highly aggressive subtype. Leukemia 15, 1785–1791 (2001). https://doi.org/10.1038/sj.leu.2402272

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