Diagnosis and Treatment of Primary Cutaneous B-Cell Lymphomas: State of the Art and Perspectives

Primary cutaneous B-cell lymphomas are rare entities that develop primarily in the skin. They constitute a heterogeneous group that represents around a quarter of primary cutaneous lymphomas. The 2018 update of the World Health Organization-European Organization for Research and Treatment of Cancer...

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Main Authors: Maëlle Dumont, Maxime Battistella, Caroline Ram-Wolff, Martine Bagot, Adèle de Masson
Format: Article
Language:English
Published: MDPI AG 2020-06-01
Series:Cancers
Subjects:
Online Access:https://www.mdpi.com/2072-6694/12/6/1497
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author Maëlle Dumont
Maxime Battistella
Caroline Ram-Wolff
Martine Bagot
Adèle de Masson
author_facet Maëlle Dumont
Maxime Battistella
Caroline Ram-Wolff
Martine Bagot
Adèle de Masson
author_sort Maëlle Dumont
collection DOAJ
description Primary cutaneous B-cell lymphomas are rare entities that develop primarily in the skin. They constitute a heterogeneous group that represents around a quarter of primary cutaneous lymphomas. The 2018 update of the World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification differentiates primary cutaneous marginal zone lymphoma and primary cutaneous follicle center lymphoma with an indolent course from primary cutaneous diffuse large B-cell lymphoma, leg type with an aggressive behavior. The broad spectrum of clinical presentations and the disease course marked by frequent relapses are diagnostic and therapeutic challenges. The classification of these diseases has been refined in recent years, which allows to better define their immunopathogenesis and specific management. In the present article, we review the main clinico-biological characteristics and the current therapeutic options of these three main subsets. Based on the recent therapeutic advances in nodal B-cell lymphomas, we focus on the development of novel treatment options applicable to primary cutaneous B-cell lymphomas, including targeted therapies, combination treatments and immunotherapeutic approaches, and cover basic, translational and clinical aspects aiming to improve the treatment of cutaneous B-cell lymphomas.
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spelling doaj.art-740290a64161413aa8e22339b9c32fdc2023-11-20T03:11:54ZengMDPI AGCancers2072-66942020-06-01126149710.3390/cancers12061497Diagnosis and Treatment of Primary Cutaneous B-Cell Lymphomas: State of the Art and PerspectivesMaëlle Dumont0Maxime Battistella1Caroline Ram-Wolff2Martine Bagot3Adèle de Masson4Department of Dermatology, APHP, Saint-Louis Hospital, F-75010 Paris, FranceINSERM U976, Human Immunology, Pathophysiology and Immunotherapy, Institut de Recherche Saint-Louis, F-75010 Paris, FranceDepartment of Dermatology, APHP, Saint-Louis Hospital, F-75010 Paris, FranceDepartment of Dermatology, APHP, Saint-Louis Hospital, F-75010 Paris, FranceDepartment of Dermatology, APHP, Saint-Louis Hospital, F-75010 Paris, FrancePrimary cutaneous B-cell lymphomas are rare entities that develop primarily in the skin. They constitute a heterogeneous group that represents around a quarter of primary cutaneous lymphomas. The 2018 update of the World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification differentiates primary cutaneous marginal zone lymphoma and primary cutaneous follicle center lymphoma with an indolent course from primary cutaneous diffuse large B-cell lymphoma, leg type with an aggressive behavior. The broad spectrum of clinical presentations and the disease course marked by frequent relapses are diagnostic and therapeutic challenges. The classification of these diseases has been refined in recent years, which allows to better define their immunopathogenesis and specific management. In the present article, we review the main clinico-biological characteristics and the current therapeutic options of these three main subsets. Based on the recent therapeutic advances in nodal B-cell lymphomas, we focus on the development of novel treatment options applicable to primary cutaneous B-cell lymphomas, including targeted therapies, combination treatments and immunotherapeutic approaches, and cover basic, translational and clinical aspects aiming to improve the treatment of cutaneous B-cell lymphomas.https://www.mdpi.com/2072-6694/12/6/1497cutaneous B-cell lymphomaslymphoid malignanciesB-cellslymphocytesskinlymphomas
spellingShingle Maëlle Dumont
Maxime Battistella
Caroline Ram-Wolff
Martine Bagot
Adèle de Masson
Diagnosis and Treatment of Primary Cutaneous B-Cell Lymphomas: State of the Art and Perspectives
Cancers
cutaneous B-cell lymphomas
lymphoid malignancies
B-cells
lymphocytes
skin
lymphomas
title Diagnosis and Treatment of Primary Cutaneous B-Cell Lymphomas: State of the Art and Perspectives
title_full Diagnosis and Treatment of Primary Cutaneous B-Cell Lymphomas: State of the Art and Perspectives
title_fullStr Diagnosis and Treatment of Primary Cutaneous B-Cell Lymphomas: State of the Art and Perspectives
title_full_unstemmed Diagnosis and Treatment of Primary Cutaneous B-Cell Lymphomas: State of the Art and Perspectives
title_short Diagnosis and Treatment of Primary Cutaneous B-Cell Lymphomas: State of the Art and Perspectives
title_sort diagnosis and treatment of primary cutaneous b cell lymphomas state of the art and perspectives
topic cutaneous B-cell lymphomas
lymphoid malignancies
B-cells
lymphocytes
skin
lymphomas
url https://www.mdpi.com/2072-6694/12/6/1497
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AT martinebagot diagnosisandtreatmentofprimarycutaneousbcelllymphomasstateoftheartandperspectives
AT adeledemasson diagnosisandtreatmentofprimarycutaneousbcelllymphomasstateoftheartandperspectives