Mantle cell lymphoma: update on diagnosis and treatment

Abstract

Mantle cell lymphoma (MCL) is a rare disease of lymphoid system arising from mature B lymphocytes with a typical translocation (11,14)(q13;q32) resulting in cyclin D1 overexpression in majority of patients. The disease is incurable and characterized by a multiple relapsing and aggressive course. At the time of diagnosis disease is usually in advanced stage with a frequent extranodal involvement in the majority of patients, and requires systemic treatment. Currently, no standard for first or second line treatment is available. The common first line treatment strategy in advanced disease is aggressive therapy tailored to the patient’s age and general health with a consolidation of high-dose chemotherapy, with or without total body irradiation (TBI) and autologous hematopoietic stem cell transplantation (auto-HSCT) in the first complete or partial remission. Treatment of relapse is essentially palliative except for limited cases of allotransplantation (allo-HSCT) burdened with high risk of treatment related mortality. Many research groups work on defining biomarkers that would prove useful in selecting appropriate therapy. Prolongation of survival seen in recent years is apparently related to more intensive therapy and use of monoclonal antibodies. New molecular targeted therapies currently arriving into clinical trials hold much of the promise for the future.
Hematologia 2010; 1, 4: 330-341