原发纵隔大B细胞淋巴瘤的诊疗进展

doi:10.16138/j.1673-6087.2025.06.11

摘要/Abstract

摘要：

原发纵隔大B细胞淋巴瘤（primary mediastinal large B-cell lymphoma, PMBCL）是一种起源于胸腺B细胞的侵袭性非霍奇金淋巴瘤，其典型表现为前纵隔肿块快速增大，常伴有胸闷、气促等局部症状和发热、盗汗等全身症状。R-CHOP（利妥昔单抗＋环磷酰胺＋多柔比星＋长春新碱＋泼尼松）和R-EPOCH（依托泊苷+泼尼松+长春新碱+环磷酰胺+多柔比星+利妥昔单抗）是常用的一线治疗方案，制定精确诊疗策略需建立在对其独特临床、病理及分子特征的全面认识之上。本文就PMBCL的临床特点、病理学特征、分子机制、鉴别诊断及最新治疗进展作一综述，旨在为疾病精确诊断、优化患者管理和提升治疗效果提供参考。

关键词: 原发纵隔大B细胞淋巴瘤, 淋巴瘤, 大B细胞淋巴瘤, 病理诊断, CHOP

Abstract:

Primary mediastinal large B-cell lymphoma (PMBCL) is an aggressive non-Hodgkin lymphoma originating from thymic B cells. It typically presents as a rapidly growing anterior mediastinal mass, often accompanied by local symptoms such as chest tightness and shortness of breath, as well as systemic symptoms like fever and night sweats. R-CHOP (rituximab + cyclophosphamide + doxorubicin + vincristine + prednisone) and R-EPOCH (etoposide + prednisone + vincristine + cyclophosphamide + doxorubicin + rituximab) are the commonly used first-line treatments. Formulating precise diagnostic and therapeutic strategies requires a comprehensive understanding of its unique clinical, pathological, and molecular characteristics. This review delivers a comprehensive overview of the clinical features, pathological characteristics, molecular mechanisms, differential diagnosis, and recent advances in treatment of PMBCL, aiming to provide a reference for accurate diagnosis, optimized patient management, and improved therapeutic outcomes.

Key words: Primary mediastinal large B-cell lymphoma, Lymphoma, Large B-cell lymphoma, Pathological diagnosis, CHOP

中图分类号:

R733

蔡璐, 易红梅, 王朝夫. 原发纵隔大B细胞淋巴瘤的诊疗进展[J]. 内科理论与实践, 2025, 20(06): 490-494.

CAI Lu, YI Hongmei, WANG Chaofu. Progress in diagnosis and treatment of primary mediastinal large B-cell lymphoma[J]. Journal of Internal Medicine Concepts & Practice, 2025, 20(06): 490-494.

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