Mantle cell lymphoma. 2012

Sergio Cortelazzo, and Maurilio Ponzoni, and Andrés J M Ferreri, and Martin Dreyling
Hematology and Bone Marrow Transplantation Unit, Azienda Ospedaliera Bolzano, Italy.

MCL is a well-characterized clinically aggressive lymphoma with a poor prognosis. Recent research findings have slightly improved the outcome of this neoplasm. The addition of rituximab to conventional chemotherapy has increased overall response rates, but it does not improve overall survival with respect to chemotherapy alone. The use of intensive frontline therapies including rituximab and consolidated by ASCT ameliorates response rate and prolongs progression-free survival, but any impact on survival remains to be proven. Furthermore, the optimal timing, cytoreductive regimen and conditioning regimen, and the clinical implications of achieving a disease remission even at molecular level remain to be elucidated. The development of targeted therapies as the consequence of better dissection of pathogenetic pathways in MCL might improve the outcome of conventional chemotherapy in most patients and spare the toxicity of intense therapy in a minority of MCL patients characterized by a relatively indolent disease. Patients not eligible for intensive regimens, such as hyperC-VAD, may be considered for less demanding therapies, such as the combination of rituximab either with CHOP or with purine analogues, or bendamustine. Allogeneic SCT can be an effective option for relapsed disease in patients who are fit enough and have a compatible donor. Maintenance rituximab may be considered after response to immunochemotherapy for relapsed disease, although there are currently no data to recommend this approach as the first-line strategy. As the optimal approach to the management of MCL is still evolving, it is critical that these patients be enrolled in clinical trials to identify better treatment options.

UI MeSH Term Description Entries
D009367 Neoplasm Staging Methods which attempt to express in replicable terms the extent of the neoplasm in the patient. Cancer Staging,Staging, Neoplasm,Tumor Staging,TNM Classification,TNM Staging,TNM Staging System,Classification, TNM,Classifications, TNM,Staging System, TNM,Staging Systems, TNM,Staging, Cancer,Staging, TNM,Staging, Tumor,System, TNM Staging,Systems, TNM Staging,TNM Classifications,TNM Staging Systems
D011379 Prognosis A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations. Prognostic Factor,Prognostic Factors,Factor, Prognostic,Factors, Prognostic,Prognoses
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D020522 Lymphoma, Mantle-Cell A form of non-Hodgkin lymphoma having a usually diffuse pattern with both small and medium lymphocytes and small cleaved cells. It accounts for about 5% of adult non-Hodgkin lymphomas in the United States and Europe. The majority of mantle-cell lymphomas are associated with a t(11;14) translocation resulting in overexpression of the CYCLIN D1 gene (GENES, BCL-1). Lymphocytic Lymphoma, Diffuse, Poorly-Differentiated,Lymphoma, Centrocytic Small-Cell,Lymphoma, Lymphocytic, Diffuse, Intermediate Differentiated,Lymphoma, Lymphocytic, Diffuse, Poorly-Differentiated,Lymphoma, Lymphocytic, Intermediate,Lymphoma, Small-Cell, Centrocytic,Mantle-Cell Lymphoma,Mantle-Zone Lymphoma,Diffuse Lymphocytic Lymphoma, Poorly-Differentiated,Lymphocytic Lymphoma, Diffuse, Poorly Differentiated,Centrocytic Small-Cell Lymphoma,Centrocytic Small-Cell Lymphomas,Diffuse Lymphocytic Lymphoma, Poorly Differentiated,Lymphoma, Centrocytic Small Cell,Lymphoma, Mantle Cell,Lymphoma, Mantle-Zone,Lymphomas, Centrocytic Small-Cell,Lymphomas, Mantle-Cell,Lymphomas, Mantle-Zone,Mantle Cell Lymphoma,Mantle Zone Lymphoma,Mantle-Cell Lymphomas,Mantle-Zone Lymphomas,Small-Cell Lymphoma, Centrocytic,Small-Cell Lymphomas, Centrocytic

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