ABSTRACT
Introduction 1148 Clinical presentation 1148 Prognostic factors 1148 Clinical prognostic factors 1149 Biological prognostic factors 1149 Clinical management 1149 Radiation in early stages 1149
Chemotherapeutic approaches 1149 Consolidation 1152 Allogeneic transplantation 1153 New innovative approaches 1153 Summary and perspectives 1153 Key points 1154 References 1154
Mantle cell lymphoma (MCL) is molecularly characterized by the chromosomal translocation t(11;14)(q13;q32), which results in a juxtaposition of the BCL1 gene locus to the immunoglobulin (Ig) heavy chain promoter and the subsequent overexpression of the cell cycle regulator protein cyclin D1. Accordingly, MCL has been recognized as a distinct subentity of non-Hodgkin lymphoma (NHL) in the previous Revised European-American Lymphoma (REAL) and the current World Health Organization (WHO) classification.1,2 The incidence of MCL is approximately 2-3/100 000 per year, representing only 5-10 percent of all NHL cases.3-5 The disease is diagnosed mainly in advanced Ann Arbor stages III/IV and characterized by an aggressive clinical course and poor prognosis with virtually no long-term survivors.6 Conventional chemotherapy has failed to substantially alter the natural course of the disease and remains a palliative approach. However, several randomized trials clearly demonstrated recently the superiority of a combined immunochemotherapy containing the anti-CD20 antibody rituximab in first-line therapy as well as in relapsed disease.7,8 In addition, a randomized trial of the European MCL Network showed that myeloablative radiochemotherapy followed by autologous stem cell transplantation significantly improved the progressionfree survival in patients up to 65 years of age as compared to conventional therapy.9 However, even after such an intensified approach, the vast majority of patients with MCL will eventually relapse. Thus, new strategies such as
or radiolabeled antibodies) or allogeneic transplantation after dose-reduced conditioning (‘mini-transplantation’) are currently being investigated to improve the dismal prognosis of MCL.
