原发性纵隔大B细胞淋巴瘤

Primary mediastinal large B-cell lymphoma.

作者信息

Martelli Maurizio, Ferreri Andrés, Di Rocco Alice, Ansuinelli Michela, Johnson Peter W M

机构信息

Department of Cellular Biotechnologies and Hematology, Sapienza University, Rome, Italy.

San Raffaele Scientific Institute, Milan, Italy.

出版信息

Crit Rev Oncol Hematol. 2017 May;113:318-327. doi: 10.1016/j.critrevonc.2017.01.009. Epub 2017 Jan 21.

DOI:10.1016/j.critrevonc.2017.01.009

PMID:28318892

Abstract

Primary mediastinal large B-cell lymphoma (PMLBCL) is a distinct clinical and biological disease from other types of DLBCL. It is more frequent in young female and constitutes 6%-10% of all DLBCL. PMLBCL is characterized by a diffuse proliferation of medium to large B-cells associated with sclerosis. Molecular analysis shows it to be a distinct entity from other DLBCL. Rituximab CHOP/MACOP-B-like regimens followed by mediastinal radiotherapy (RT) were associated with a 5-years PFS of 75%-85%. More intensive regimens, as DA-EPOCH-R without mediastinal RT, have shown very promising results, but this therapeutic advance needs to be confirmed in further prospective trials. The role of consolidative mediastinal RT should be still better assess in prospective comparative studies. PET-CT scan is a powerful tool to define the real quality of response and it is hoped that future prospective trials may allow its role in the de-escalation of mediastinal RT.

摘要

原发性纵隔大B细胞淋巴瘤（PMLBCL）是一种在临床和生物学特性上与其他类型弥漫性大B细胞淋巴瘤（DLBCL）不同的疾病。它在年轻女性中更为常见，占所有DLBCL的6%-10%。PMLBCL的特征是中到大B细胞的弥漫性增殖并伴有硬化。分子分析表明它是一种与其他DLBCL不同的实体。利妥昔单抗CHOP/MACOP-B样方案联合纵隔放疗（RT）后的5年无进展生存率（PFS）为75%-85%。更强化的方案，如不含纵隔放疗的DA-EPOCH-R，已显示出非常有前景的结果，但这一治疗进展需要在进一步的前瞻性试验中得到证实。巩固性纵隔放疗的作用仍应在前瞻性比较研究中进行更好的评估。PET-CT扫描是确定实际缓解质量的有力工具，希望未来的前瞻性试验能够明确其在纵隔放疗降阶梯治疗中的作用。