Boleti E, Johnson P W M
Cancer Research UK Clinical Centre, Southampton General Hospital, Southampton, UK.
Hematol Oncol. 2007 Dec;25(4):157-63. doi: 10.1002/hon.818.
Primary mediastinal B-cell lymphoma (PMBCL) is a sub-type of the heterogeneous diffuse large B-cell lymphoma category, and comprises approximately 5% of all non-Hodgkin's lymphomas (NHL). It was first recognized as a distinct clinico-pathologic entity 20 years ago, and recent work has further characterized specific molecular features. Gene expression profiling has suggested a partial overlap with nodular sclerosing Hodgkin lymphoma (HL), with which it shares some clinical features. The optimal management remains a matter of debate. There is uncertainty as to whether weekly alternating chemotherapy regimens may be more effective than CHOP, whether consolidation radiotherapy (RT) to the mediastinum is always required, whether PET scanning can be used to determine this, and whether the use of rituximab as part of initial therapy will change the answers to these questions. The International Extranodal Lymphoma Study Group (IELSG) 26 clinicopathologic study of PMBCL, which has recently opened, represents a first attempt to gather data prospectively on some of these issues.
原发性纵隔B细胞淋巴瘤（PMBCL）是异质性弥漫大B细胞淋巴瘤类别的一个亚型，约占所有非霍奇金淋巴瘤（NHL）的5%。20年前它首次被确认为一种独特的临床病理实体，近期的研究进一步明确了其特定的分子特征。基因表达谱分析表明它与结节硬化型霍奇金淋巴瘤（HL）有部分重叠，二者有一些共同的临床特征。最佳治疗方案仍存在争议。关于每周交替化疗方案是否比CHOP更有效、纵隔是否总是需要巩固放疗（RT）、PET扫描能否用于确定这一点以及利妥昔单抗作为初始治疗的一部分是否会改变这些问题的答案，目前仍不确定。国际结外淋巴瘤研究组（IELSG）最近启动的关于PMBCL的26号临床病理研究，是首次尝试前瞻性地收集有关其中一些问题的数据。
