Treatment of mantle cell lymphomas: recommendations of the Belgian Hematological Society

Mantle cell lymphoma was recognised in the nineties and is characterised by the t(11;14)(q13;q32) translocation which results in overexpression of cyclin D1.1 This disease represents approximately 6% of all non-Hodgkin's lymphomas. Mantle cell lymphoma generally affects patients over 60 years-old. Most patients have advanced disease (>70 % Ann Arbor stage IV). Several efforts have been made to predict outcome in mantle cell lymphoma. The cell-proliferation marker Ki-67, the Mantle Cell Lymphoma International Prognostic Index, fluorodeoxyglucose positron emission tomography and minimal residual disease are prognostic tools. For young patients, chemoimmunotherapy followed by high-dose chemotherapy plus stem cell transplantation is the treatment of choice. For the main group of older patients, chemo-immunotherapy followed by maintenance with rituximab is the gold standard. In relapses, temsirolimus is actually registered and new drugs, such as ibrutinib, are currently evaluated with promising preliminary results.