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Blood, 20 August 2009, Vol. 114, No. 8, pp. 1469-1476. Prepublished online as a Blood First Edition Paper on June 25, 2009; DOI 10.1182/blood-2009-02-179739.
Submitted February 6, 2009
Oncology Institute of Southern Switzerland, Bellinzona, Switzerland * Corresponding author; email: michele.ghielmini{at}eoc.ch.
Mantle cell lymphoma (MCL) is included in the WHO classification as distinct lymphoma subtype characterized by the t(11;14)(q13;q32) translocation, which results in over-expression of Cyclin D1. The clinical presentation often includes extra-nodal involvement, particularly of the bone marrow and gut. The prognosis of patients with MCL (median OS 3-5 years) is the poorest among B cell lymphoma patients, even though a prospectively difficult to identify sub-group can survive for years with little or no treatment. Conventional chemotherapy is not curative but obtains frequent remissions (60-90%) which are usually shorter (1-2 years) compared to other lymphoma entities. Very intensive regimens, comprising autologous and allogeneic stem cell transplantation seem required to improve the outcome but, the median age of diagnosis being 60 years or more, such approaches are feasible only in a limited proportion of patients. The possibility to treat patients based on prognostic factors needs to be investigated prospectively
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