Igor Aurer1. 1. Division of Hematology, Department of Internal Medicine, University Hospital Centre Zagreb and Medical School, University of Zagreb, Zagreb Croatia.
Abstract
PURPOSE OF REVIEW: Mantle cell lymphoma (MCL) is a disease predominantly affecting elderly patients with bad prognosis. Recently, a number of new agents have been shown to be active in this disease. This article reviews this data from the standpoint of everyday practice. RECENT FINDINGS: Front-line regimens combining rituximab with CHOP, cytarabine, bendamustine or lenalidomide, frequently followed by rituximab maintenance, remain the standard. Choice depends on the aggressiveness of the disease, patient characteristics and local availability. BTK inhibitors have emerged as most important agents for the treatment of relapsed/refractory disease, but many other options exist, including rituximab, chemotherapy, immunomodulators, bortezomib and venetoclax that can be used in combination and sequentially. In frail patients, combinations of rituximab with low-intensity chemotherapy, immunomodulators and BTK inhibitors can be useful but care must be taken to avoid additive drug toxicities and interaction. SUMMARY: Recent advances in treatment of MCL enable the delivery of multiple lines of therapy resulting in prolonged survival in most patients. Results of treatment of blastoid MCL with high Ki67 remain unsatisfactory and are an unmet medical need.
PURPOSE OF REVIEW: Mantle cell lymphoma (MCL) is a disease predominantly affecting elderly patients with bad prognosis. Recently, a number of new agents have been shown to be active in this disease. This article reviews this data from the standpoint of everyday practice. RECENT FINDINGS: Front-line regimens combining rituximab with CHOP, cytarabine, bendamustine or lenalidomide, frequently followed by rituximab maintenance, remain the standard. Choice depends on the aggressiveness of the disease, patient characteristics and local availability. BTK inhibitors have emerged as most important agents for the treatment of relapsed/refractory disease, but many other options exist, including rituximab, chemotherapy, immunomodulators, bortezomib and venetoclax that can be used in combination and sequentially. In frail patients, combinations of rituximab with low-intensity chemotherapy, immunomodulators and BTK inhibitors can be useful but care must be taken to avoid additive drug toxicities and interaction. SUMMARY: Recent advances in treatment of MCL enable the delivery of multiple lines of therapy resulting in prolonged survival in most patients. Results of treatment of blastoid MCL with high Ki67 remain unsatisfactory and are an unmet medical need.