Immune pathways, current and potential therapies in Mycosis fungoides and Sezary syndrome.

IF 3.9 3区 医学 Q2 IMMUNOLOGY
Friyana K Bhabha, Carrie Van Der Weyden, Joshua M L Casan, Belinda A Campbell, Christopher McCormack, H Miles Prince
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Abstract

Introduction: Primary cutaneous T cell lymphomas (CTCL) comprise a diverse group of malignancies with distinct and variable treatment options and prognoses. Differentiating between subtypes can be challenging due to overlapping heterogeneous clinical and histopathologic features, mandating careful clinicopathologic correlation for diagnosis. Mycosis fungoides (MF) is the most common subtype, whereas the less frequent Sézary syndrome (SS) is viewed at the more aggressive end of the MF/SS spectrum. Large cell transformation (LCT) is a rare phenomenon associated with poor prognosis, arising in a subset of patients with MF/SS, although the exact etiology and molecular pathogenesis remains unclear.

Areas covered: Progression of these diseases is influenced by a variety of immunologic factors. Our advancing understanding of immune pathways and tumor microenvironment may accelerate the development of targeted therapies. This review examines the immune-modulating effects of current and emerging therapeutic drugs for MF/SS. It encompasses both established clinical guidelines and emerging targeted agents currently under investigation in clinical trials.

Expert opinion: The treatment landscape for CTCL, especially advanced disease, is becoming increasingly focused on immunotherapies and biologic agents. These treatments have the potential to provide patients with more effective clinical outcomes. The development of synergistic combination therapy will also be important expanding therapeutic options in patients with advanced CTCL.

蕈样真菌病和癫痫综合征的免疫途径、目前和潜在的治疗方法。
原发性皮肤T细胞淋巴瘤(CTCL)包括一组不同的恶性肿瘤,具有独特和可变的治疗选择和预后。由于重叠的异质临床和组织病理学特征,区分亚型可能具有挑战性,需要仔细的临床病理相关性进行诊断。蕈样真菌病(MF)是最常见的亚型,而不太常见的ssamzary综合征(SS)则被视为MF/SS谱系中更具侵袭性的一端。大细胞转化(LCT)是一种罕见的与预后不良相关的现象,发生在MF/SS患者的一个亚群中,尽管确切的病因和分子发病机制尚不清楚。涵盖领域:这些疾病的进展受到多种免疫因素的影响。我们对免疫途径和肿瘤微环境的深入了解可能会加速靶向治疗的发展。本文综述了目前和新兴的MF/SS治疗药物的免疫调节作用。它既包括已建立的临床指南,也包括目前正在临床试验中研究的新兴靶向药物。专家意见:CTCL的治疗前景,特别是晚期疾病,越来越多地集中于免疫疗法和生物制剂。这些治疗方法有可能为患者提供更有效的临床结果。协同联合治疗的发展也将是扩大晚期CTCL患者治疗选择的重要因素。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
CiteScore
7.60
自引率
2.30%
发文量
221
审稿时长
6-12 weeks
期刊介绍: Expert Review of Clinical Immunology (ISSN 1744-666X) provides expert analysis and commentary regarding the performance of new therapeutic and diagnostic modalities in clinical immunology. Members of the International Editorial Advisory Panel of Expert Review of Clinical Immunology are the forefront of their area of expertise. This panel works with our dedicated editorial team to identify the most important and topical review themes and the corresponding expert(s) most appropriate to provide commentary and analysis. All articles are subject to rigorous peer-review, and the finished reviews provide an essential contribution to decision-making in clinical immunology. Articles focus on the following key areas: • Therapeutic overviews of specific immunologic disorders highlighting optimal therapy and prospects for new medicines • Performance and benefits of newly approved therapeutic agents • New diagnostic approaches • Screening and patient stratification • Pharmacoeconomic studies • New therapeutic indications for existing therapies • Adverse effects, occurrence and reduction • Prospects for medicines in late-stage trials approaching regulatory approval • Novel treatment strategies • Epidemiological studies • Commentary and comparison of treatment guidelines Topics include infection and immunity, inflammation, host defense mechanisms, congenital and acquired immunodeficiencies, anaphylaxis and allergy, systemic immune diseases, organ-specific inflammatory diseases, transplantation immunology, endocrinology and diabetes, cancer immunology, neuroimmunology and hematological diseases.
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