Mantle cell lymphoma

Mantle cell lymphoma (MCL) is a subtype of non-Hodgkin lymphoma characterized by the pathognomonic chromosomal translocation t(11;14)(q13;q32) leading to constitutive cyclin D1 overexpression. Despite high initial response rates, early relapses and rapid disease progression occur frequently after conventional chemotherapy, resulting in a median survival of only 3–5 years. However, 10–15% of patients present with a more indolent, chronic course. Dose-intensified treatment regimens containing cytarabine, rituximab, and autologous stem cell transplantation represent the current standard for young and fit patients and can achieve long-term remissions. For the majority of elderly patients, rituximab maintenance therapy can result in prolonged survival. With the BTK inhibitor ibrutinib, the immunomodulatory drug lenalidomide, the MTOR inhibitor temsirolimus, and the proteasome inhibitor bortezomib, highly efficacious targeted therapies have been approved for the treatment of relapsed or refractory MCL, alone or in combination with chemotherapy. Bortezomib combined with rituximab, cyclophosphamide, doxorubicin, and prednisolone (VR-CAP) now represents the new standard for the treatment of elderly patients. Implementation of the targeted agents in multimodal treatment plans also for first-line therapy and the identification of prognostically relevant genetic varieties and potential new targets in MCL to further optimize MCL treatment are under intensive investigation.