Mantle cell lymphoma—Update on molecular biology, prognostication and treatment approaches

Abstract

Mantle cell lymphoma (MCL) is clinically characterized by its heterogenous behavior with courses ranging from indolent cases that do not require therapy for years to highly aggressive MCL with very limited prognosis. The development and implementation of new targeted and immunotherapeutic approaches have already improved therapeutic options especially for refractory or relapsed disease. Nevertheless, to further optimize MCL treatment, early identification of individual risk profile and risk‐adapted, patient‐tailored choice of therapeutic strategy needs to be prospectively incorporated in clinical patient management. This review summarizes the current knowledge and standard of care regarding biology and clinical management of MCL, highlighting the implementation of new therapeutic approaches especially targeting the immune system.

Survival Outcomes of Patients with Mantle Cell Lymphoma: A Retrospective, 15-Year, Real-Life Study

“…We investigated the survival outcome of MCL patients over a 15-year treatment interval, between 2006 and 2020. MCL prognosis has significantly improved in recent years; however, the possible long-term survival benefit of new treatment options has not been clearly established outside of clinical trials [ 1 , 2 , 3 , 4 ].…”

“…According to current guidelines, first-line treatment should include rituximab, HD cytarabine-containing regimens, and ASCT as consolidation for younger, chemosensitive patients, while older patients should receive chemoimmunotherapy, such as BR, R-BAC, R-CHOP, and VR-CAP [ 1 , 2 , 3 , 4 ]. The recently published, long-term follow-up of the R-BAC regimen in older, newly diagnosed MCL patients demonstrated sustained efficacy over time.…”

“…Mantle cell lymphoma (MCL) represents a rare subtype of non-Hodgkin lymphoma (NHL), characterized by cyclin D1 overexpression, that is associated with t(11;14)(q13;q32) translocation [ 1 , 2 , 3 , 4 ]. MCL incidence has increased over the last few years, especially among elderly patients [ 2 , 4 ].…”

“…MCL incidence has increased over the last few years, especially among elderly patients [ 2 , 4 ]. MCL is commonly diagnosed as advanced-stage, with frequent nodal, extranodal, and bone marrow involvement, and it is divided into classical, pleomorphic, and blastoid variants, with an aggressive disease course in most cases [ 1 , 2 , 3 , 4 ]. However, 10–20% of patients are characterized by a leukemic, non-nodal (LNN) presentation, with more indolent clinical behavior and prolonged survival [ 4 , 5 , 6 ].…”

The Role of Transglutaminase 2 in Cancer: An Update

“…TG2 is also important in Mantle Cell Lymphoma (MCL), which is an incurable lymphoma originating from the mantle zone of the lymph node (where memory and naïve B cells and T cells can be found) [ 102 ]. In this neoplasm, TG2 expression leads to the constitutive activation of NF-κB and consequent chemoresistance [ 103 ].…”