EHA-SWG 2017 | Rare Lymphomas: current and future treatment of MCL

On March 10–12 2017, the EHA-SWG meeting on Rare Lymphomas took place in Barcelona, Spain, and was jointly chaired by Prof. Martin Dreyling, from Klinikum der Universität München, Germany, and Prof. Marie José Kersten, from the Academic Medical Center, Amsterdam, The Netherlands.

On March 10^th 2017, M. Dreyling gave a talk titled “Current standards and future studies”, during the “Mantle Cell Lymphoma” scientific session.

Prof. Dreyling began by explaining that MCL is characterized by the t(11;14)(q13;q32) translocation, which results in constitutional overexpression of cyclin D1 and dysregulation of the cell cycle in the majority of cases.

MCL shows a heterogeneous, but mostly aggressive, clinical course. The disease also displays a continuous relapse pattern and has a median survival of approximately 3–5 years. Recently, there have been reports describing an indolent disease course in ~10% of cases which have improved outcome.

Elderly patients

• Bendamustine is the preferred choice of chemotherapy in more indolent cases (based on LDH and Ki-67) because of its favorable toxicity profile
• In highly proliferating cases, regimens containing cytarabine are more appropriate
• Maintenance with rituximab was reported to significantly improve survival after R-CHOP in a large, European, phase III trial

Younger patients

• Significantly improved time to treatment failure reported when younger patients are treated with cytarabine-containing induction followed by myeloablative conditioning and ASCT
• Subsequent maintenance with rituximab improves PFS and OS further

Targeted therapies

• Small inhibitors of BCR pathway: e.g. the BTK inhibitor ibrutinib has favorable toxicity profile and induces long-term remissions; however, aggressive early relapses have been reported
• Lenalidomide has shown superiority to mono-chemotherapy in a randomized, phase III trial

• Temsirolimus has shown superiority to chemotherapy, but appears even more effective in combination with chemotherapy (such as the BeRT regime)

• In first-line, bortezomib (proteasome inhibitor) plus CHOP-like therapy has been reported to be superior to conventional R-CHOP

Prof. Dreyling concluded the talk by stating that current trials are exploring multimodal approaches involving the aforementioned, as well as other, molecular targeted approaches: we are entering “the era of combinations”.

Based on a patient’s individual molecular risk profile, clinical presentation, and comorbidities, in the future we will be able to draw from an armamentarium of numerous therapeutic approaches to individualize therapy.