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Annals of Oncology Advance Access published online on October 24, 2007

Annals of Oncology, doi:10.1093/annonc/mdm447
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© 2007 European Society for Medical Oncology

Central nervous system involvement in mantle cell lymphoma

A. Ferrer1,{dagger}, F. Bosch1, N. Villamor3, M. Rozman3, F. Graus2, G. Gutiérrez1, S. Mercadal1, E. Campo3, C. Rozman1, A. López-Guillermo1,* and E. Montserrat1

1 Department of Hematology
2 Department of Neurology and
3 Hematopathology Unit, Hospital Clínic, Postgraduate School of Hematology ‘Farreras Valentí’, Institut d'Investigacio Biomedica ‘August Pi i Sunyer’, Barcelona, Spain

* Correspondence to: Dr A. López-Guillermo, Department of Hematology, Hospital Clínic, Villarroel, 170, 08036 Barcelona, Spain. Tel: +34-932275575; Fax: +34-932275484; E-mail: alopezg{at}clinic.ub.es

Background: Extranodal involvement, including central nervous system (CNS), is a frequent event in patients with mantle cell lymphoma (MCL). However, the incidence, risk factors, and impact on outcome remain controversial.

Patients and methods: Main clinical, biological, and evolutive features of 82 patients (60 males/22 females; median age: 61 years) diagnosed with MCL (blastoid, 26%) in a single institution were analyzed for risk of CNS involvement and prognosis.

Results: Most patients had advanced stage and intermediate or high-risk International Prognostic Index (IPI). Eleven patients eventually developed CNS involvement with an actuarial 5-year risk of 26% (95% confidence interval 10% to 42%). In one asymptomatic patient, cerebrospinal fluid infiltration was detected at staging maneuvers (1/62; 1.6%). The remaining 10 patients developed neurological symptoms during the course of the disease (median time from diagnosis, 25 months). Initial variables predicting CNS involvement were blastoid histology, high proliferative index measured by Ki-67 staining, high lactate dehydrogenase (LDH) and intermediate- or high-risk IPI. Histological subtype and serum LDH maintained significance in multivariate analysis. Treatment of CNS infiltration consisted of intrathecal chemotherapy (two cases), and intrathecal chemotherapy plus systemic treatment (seven cases). Median survival after CNS involvement was 4.8 months, patients with this complication having shorter survival than those with no CNS disease.

Conclusion: This study confirms the high incidence of CNS involvement in MCL patients. Treatments aimed at preventing this complication are warranted.

central nervous system involvement, mantle cell lymphoma, prognosis, risk factors


{dagger} Present address: Laboratori de Citologia Hematològica, Department of Pathology, Hospital del Mar, Barcelona, Spain

Received for publication May 17, 2007. Revision received August 9, 2007. Accepted for publication August 13, 2007.


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