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Inicio Medicina Clínica (English Edition) Mantle cell lymphoma: Towards a personalized therapeutic strategy?
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Vol. 144. Issue 12.
Pages 553-559 (June 2015)
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Vol. 144. Issue 12.
Pages 553-559 (June 2015)
Review
Mantle cell lymphoma: Towards a personalized therapeutic strategy?
Linfoma de células del manto: ¿hacia una estrategia terapéutica individualizada?
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Belén Navarro Matilla, José A. García-Marco
Corresponding author
jagarciam@aehh.org

Corresponding author.
Servicio de Hematología y Hemoterapia, Hospital Universitario Puerta de Hierro-Majadahonda, Majadahonda, Madrid, Spain
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Table 1. Differential diagnosis of mantle cell lymphoma with other chronic B-cell lymphopfoliferative disease according to immunophenotypic and genetic alterations.
Table 2. International prognostic index estimation in mantle cell lymphoma which has been simplified for prognostic classification.
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Abstract

Mantle cell lymphoma (MCL) is a clinically heterogeneous non-Hodgkin lymphoma with an aggressive clinical behaviour and short survival in some cases and an indolent course in others. Advances in the biology and pathogenesis of MCL have unveiled several genes involved in deregulation of cell cycle checkpoints and the finding of subclonal populations with specific recurrent mutations (p53, ATM, NOTCH2) with an impact on disease progression and refractoriness to treatment. Prognostic stratification helps to distinguish between indolent and aggressive forms of MCL. Currently, younger fit patients benefit from more intensive front line chemotherapy regimens and consolidation with autologous transplantation, while older or frail patients are treated with less intensive regimens and rituximab maintenance. For relapsing disease, the introduction of bortezomib and lenalidomide containing regimens and B-cell receptor pathway inhibitors such as ibrutinib and idelalisib in combination with immunochemotherapy have emerged as therapeutic agents with promising clinical outcomes.

Keywords:
Mantle cell lymphoma
Treatment
Diagnosis
Resumen

El linfoma de células del manto (LM) es una entidad clínica heterogénea con un comportamiento clínico agresivo y una supervivencia corta en algunos pacientes, mientras que en otros sigue un curso clínico indolente. Los recientes avances en la biología del LM han demostrado la existencia de genes implicados en la desregulación de vías relacionadas con el ciclo celular, así como la presencia de poblaciones subclonales con mutaciones recurrentes (p53, ATM, NOTCH2) con impacto en la progresión clínica y refractariedad al tratamiento. La estratificación pronóstica ayuda a distinguir entre formas indolentes y agresivas del LM. Actualmente, los pacientes jóvenes se benefician de quimioterapia alternante y consolidación con trasplante autólogo en primera línea, y los pacientes ancianos se tratan con regímenes estándar y mantenimiento con rituximab. Los pacientes en recaída se benefician de regímenes que incluyen bortezomib y lenalidomida. Además, el empleo de inhibidores de tirosincinasas (ibrutinib, idelalisib) está ofreciendo resultados clínicos muy prometedores.

Palabras clave:
Linfoma del manto
Tratamiento
Diagnóstico

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