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原发性侵袭性口腔淋巴瘤(PAOL):诊断、分子特征、治疗策略与牙科医生及血液科医生整合作用的叙述性综述

英文原题:Primary Aggressive Oral Lymphomas (PAOL): A Narrative Review of Diagnosis, Molecular Features, Therapeutic Approaches, and the Integrated Role of Dentists and Hematologists.

查看英文原题

Primary Aggressive Oral Lymphomas (PAOL): A Narrative Review of Diagnosis, Molecular Features, Therapeutic Approaches, and the Integrated Role of Dentists and Hematologists.

PubMed 2025/09/26(内容时间) Cancers (Basel) Q2 · IF 4.8(JCR 2025)

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中文摘要

原发性侵袭性口腔淋巴瘤(PAOL)是一类罕见的结外非霍奇金淋巴瘤,发生于口腔,确诊时没有其他系统受累证据。PAOL仅占所有淋巴瘤的约2%–3%。其最常见类型为侵袭性B细胞亚型,如弥漫大B细胞淋巴瘤(DLBCL)和浆母细胞淋巴瘤(PBL),偶见伯基特淋巴瘤及T细胞/NK细胞淋巴瘤。临床表现常不具特异性,例如肿胀、疼痛、溃疡及牙齿松动,容易与良性牙科疾病混淆,导致诊断延迟。综合诊断需结合全面口腔检查、影像学检查(CT、MRI、PET),以及通过免疫组化和遗传学检测进行确诊和分期的活检。治疗通常采用全身化疗;对于CD20阳性肿瘤,常联合利妥昔单抗,局限性病变还可辅以放疗。对PAOL基因组和微环境特征的持续研究,正为改善患者结局的新型靶向疗法铺路。HIV感染者或移植患者的PAOL常与病毒合并感染(EBV、HHV-8)有关,可能需要个体化治疗,包括优化免疫状态。牙医不仅承担诊断职责,还应积极参与癌症治疗期间的预防性和支持性口腔护理,并在治疗后继续监测复发和处理慢性治疗后遗症。本综述全面介绍PAOL的流行病学、临床病理和分子特征、当前及新兴治疗方法,以及牙医与血液科医生在患者照护中的协作作用。

展开英文摘要原文

Primary aggressive oral lymphomas (PAOL) are a rare subset of extranodal non-Hodgkin lymphomas arising in the oral cavity without evidence of other systemic involvement at diagnosis. PAOL accounts for only about 2-3% of all lymphomas. They most commonly belong to aggressive B-cell subtypes such as Diffuse large B-cell lymphoma (DLBCL) and plasmablastic lymphoma (PBL), with occasional cases of Burkitt lymphoma and T-cell/NK-cell lymphomas. Clinically, these malignancies often present with non-specific symptoms (e. g. , swelling, pain, ulceration, tooth mobility) that mimic benign dental conditions, leading to diagnostic delays. An integrated diagnostic approach-combining thorough oral examination, imaging (CT, MRI, PET), and definitive biopsy with immunohistochemistry and genetic studies-is critical for accurate diagnosis and staging.

Treatment typically involves systemic chemotherapy, often combined with rituximab for CD20+ tumors and adjunctive radiotherapy for localized disease. Ongoing research into the genomic and microenvironmental landscape of PAOL is paving the way for novel targeted therapies to improve outcomes. In HIV+ or transplant patients, PAOL are often driven by viral co-infections (EBV, HHV-8) and may require tailored therapy, including optimization of immune status.

The dentist's role encompasses not only diagnosis but also active participation in cancer therapy through preventive and supportive dental care, and persists thereafter by monitoring for recurrence and treating chronic treatment sequelae. This review provides a comprehensive overview of PAOL's epidemiology, clinical-pathologic and molecular features, current and emerging treatments, and the essential collaborative role of dentists and hematologists in patient care.

论文信息

作者
Bibas M、Pilloni A、Maggio E、Antinori A、Mazzotta V
第一作者单位
Department of Clinical Research: Hematology, National Institute for Infectious Diseases "Lazzaro Spallanzani" I.R.C.S.S., 00149 Rome, Italy.Italy
通讯作者单位
Department of Clinical Research: Infectious Diseases, National Institute for Infectious Diseases "Lazzaro Spallanzani" I.R.C.S.S., 00149 Rome, Italy.Italy
文献类型
综述
期刊
Cancers2025 Sep 26
原文标识
PubMed 41097666 · DOI 10.3390/cancers17193138