Mantle lymphomas (MCL) represent a recently recognised sub-type of malignant lymphomas which are charaterised by a specific histology and immunophenotype and an unfavourable clinical course. Hence, response to chemotherapy is poor and patients with this disease usually face a median survival of less than three years. The recent discovery that MCL are associated with a specific chromosomal abnormality, the translocation t (11;14) that leads to the deregulation of cyclin D 1 and over-expression of bcl 1 and a consecutive impairment of cell cycle control has opened new perspectives to better understand the biology of this lymphoma and also potentially its clinical course. The current multinational study aims at gaining new insights into the biology of MCL and its appropriate clinical management and tries to explore a novel therapeutic strategy comprising myeloablative radio-chemotherapy with subsequent peripheral blood stem cell transplantation. These objectives are addressed in three major steps :
- The assessment of clinical and histopathologic features of presentation as obtained from a retrospective analysis of patients that have been treated with conventional therapies at the participating institutions;
- The biologic characterisation of MCL by means of modern immunophenotypic and molecular techniques;
- The prospective evaluation of myeloablative radio-chemotherapy with subsequent blood stem cell transplantation.