Vol 1, No 4 (2010)
Review paper
Published online: 2010-11-24
Mantle cell lymphoma: update on diagnosis and treatment
Hematologia 2010;1(4):330-341.
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
Hematologia 2010; 1, 4: 330-341
Keywords: mantle cell lymphomadiagnosistreatment