Mantle Cell Lymphoma – Cutting‑ edge Diagnostics and Treatment Approaches

Czech version

Authors: P. Klener Jr.; M. Trněný
Authors‘ workplace: I. interní klinika – klinika hematologie 1. LF UK a VFN v Praze
Published in: Klin Onkol 2015; 28(Supplementum 3): 80-86
doi: https://doi.org/10.14735/amko20153S80

Overview

Background:
Mantle cell lymphoma represents a specific subtype of B‑ cell non‑Hodgkin lymphoma characterized on the molecular level by translocation t(11;14)(q13;q32) leading to aberrant overexpression of cyclin D₁ and deregulation of the cell cycle. Despite sporadic indolent forms of mantle cell lymphoma, majority of patients present with advanced aggressive disease that requires immediate treatment. Despite chemosensitive nature of mantle cell lymphoma, approximately 10% patients present with a refractory disease, and the vast majority of patients who initially respond to therapy, relapse sooner or later. The course of mantle cell lymphoma thus represents a chronically relapsing malignancy requiring further and further lines of therapies. Prognosis of relapsed or refractory (R/ R) mantle cell lymphoma is dismal.

Aim:
The goal of this article is to provide a cutting‑ edge review of currently used diagnostic and treatment approaches for mantle cell lymphoma.

Results:
Several key modifications of the therapeutic algorithm of mantle cell lymphoma treatment implemented in the past 10 years resulted in significantly improved prognosis of patients. The milestones in the therapy of mantle cell lymphoma include incorporation of anti‑CD20 monoclonal antibody rituximab into induction therapy, intensification of polychemotherapeutic regimen including implementation of high‑dose cytarabine, consolidation of response with high‑dose therapy and autologous stem cell transplantation (HDT‑ ASCT) in younger fit patients, and maintenance therapy with rituximab in the elderly patients. Besides such “optimization” of front‑line therapy, introduction of novel anti‑lymphoma agents into therapy of R/ R mantle cell lymphoma also contributed (and will contribute in the future) to improved prognosis of mantle cell lymphoma. Among these agents, there is a new cytostatic drug bendamustine, Bruton tyrosine‑ kinase inhibitor ibrutinib, immunomodulatory agent lenalidomide, mTOR inhibitor temsirolimus and proteasome inhibitor bortezomib.

Conclusion:
The overall survival of mantle cell lymphoma virtually doubled in the recent 10 years as a result of two key factors: 1. optimization of front‑line therapy with “conventional” anti‑lymphoma agents, and 2. brand new possibilities of therapy for R/ R mantle cell lymphoma thanks to the introduction of novel anti‑lymphoma agents. Combinatorial approaches using most efficacious combinations of novel and conventional anti‑mantle cell lymphoma agents will definitely lead to further improvements of survival parameters in mantle cell lymphoma patients in near future.

Key words:
mantle cell lymphoma – minimal residual disease – autologous transplantation

This study was supported by project PRVOUK-27//LF1/1, IGA-MZ NT/13072-4, IGA-MZ NT 13201-4/2012.

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manuscript met the ICMJE recommendation for biomedical papers.

Submitted:
24. 9. 2015

Accepted:
27. 9. 2015

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