Onkológia 1/2024

Mantle cell lymphoma

Mantle cell lymphoma (MCL) arises from malignant transformation of mantle B-lymphocytes around the germinal center of the lymph node. Its characteristic features include that it is not curable with conventional chemotherapy, relapses after a certain interval of remission, and generally has an aggressive course of behavior, with a median overall survival (OS) of 3 years in the past. It is a heterogeneous disease that has several histological types with different prognosis and only in a small number of patients it has an indolent course that does not require immediate treatment. Today, the standard of treatment for young fit patients is complex chemoimmunotherapy, including high-dose chemotherapy with autologous hematopoietic stem cells support and the inclusion of maintenance monotherapy with monoclonal antibodies, which can prolong overall survival by 5 to 10 years. Further prolongation of PFS (progression-free survival) and OS can be achieved by knowing the tumor microenvironment, prognostic factors, gene aberrations, and signaling pathways, which leads to the development of exact targeted therapy with new molecules from groups such as: proteasome inhibitors, CDK blockers (cyclin dependent kinase), serine/threonine and tyrosine kinases, PI3K/AKT (phosphatidylinositol 3-kinase, serine/threonine AKT kinase), mTOR (mammalian target of rapamycin), HDAC (histone deacetylases), HSP90 (heat shock protein), BH3 mimetics (BCL-2 homology domain 3), IMIDs and others. New targeted drugs with a limited spectrum of side effects offer the possibility of better therapeutic results, even in the older age group ≥ 65 years and unfit patients. It is likely that their inclusion in the complex treatment of MCL will change the entire structure of the current therapeutic plan in favor of the patient with a further extension of the remission interval, PFS and OS.

Keywords: mantle cell lymphoma, chemoimunotherapy, targeted therapy, maintenance therapy, Bruton’s tyrosin kinase inhibitors