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原发性皮肤γδ T细胞淋巴瘤的临床、诊断及预后特征

Clinical, Diagnostic and Prognostic Characteristics of Primary Cutaneous Gamma Delta T-cell Lymphomas.

作者信息

Muhsen Ibrahim N, El Fakih Riad, Hamadani Mehdi, Lazarus Hillard M, Kharfan-Dabaja Mohamed A, Aljurf Mahmoud

机构信息

Department of Medicine, Houston Methodist Hospital, Houston, TX USA.

Oncology Center, King Faisal Specialist Hospital and Research Center, PO Box 3354, Riyadh, 11211 Saudi Arabia.

出版信息

Clin Hematol Int. 2022 Jun 22;4(1-2):1-10. doi: 10.1007/s44228-022-00011-9. eCollection 2022 Jun.

Abstract

Primary cutaneous T-cell lymphoma (PCGDTL) is a rare subtype of non-Hodgkin lymphoma (NHL) that arises from T-cells with T-cell receptors. The exact incidence of PCGDTL is unknown, as it is usually lumped with other cutaneous lymphomas, which are also uncommon. It is one of the peripheral T-cell lymphoma (PTCL) subtypes which is known to have a dismal prognosis due to poor response and the paucity of available therapies. Despite the rarity and uncertainties of PCGDTL, a number of studies over the past decade were published about the pathologic, diagnostic, cytogenetic and clinical features of this disease. These diagnostic advances will open the doors to explore new therapeutics for this rare entity, specifically targeted and immune therapies. In this review, we highlight these advances, summarize the contemporary treatment approaches, and shed the light on future potential therapeutic targets.

摘要

原发性皮肤T细胞淋巴瘤(PCGDTL)是非霍奇金淋巴瘤(NHL)的一种罕见亚型,起源于具有T细胞受体的T细胞。PCGDTL的确切发病率尚不清楚,因为它通常与其他同样罕见的皮肤淋巴瘤归为一类。它是外周T细胞淋巴瘤(PTCL)的亚型之一,已知由于反应不佳和可用治疗方法匮乏而预后不良。尽管PCGDTL罕见且存在不确定性,但在过去十年中发表了许多关于该疾病的病理、诊断、细胞遗传学和临床特征的研究。这些诊断进展将为探索针对这种罕见疾病的新疗法,特别是靶向疗法和免疫疗法打开大门。在本综述中,我们重点介绍这些进展,总结当代治疗方法,并阐明未来潜在的治疗靶点。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/20a9/9358781/a14fa8b1d381/44228_2022_11_Fig1_HTML.jpg

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