Abou-Elella A A, Weisenburger D D, Vose J M, Kollath J P, Lynch J C, Bast M A, Bierman P J, Greiner T C, Chan W C, Armitage J O
Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha 68198-3135, USA.
J Clin Oncol. 1999 Mar;17(3):784-90. doi: 10.1200/JCO.1999.17.3.784.
To investigate whether primary mediastinal large B-cell lymphoma (PMLBL) is a distinct clinicopathologic entity with a more aggressive course than other diffuse large B-cell lymphomas (DLBL).
All patients with CD20-positive DLBL who presented with a mediastinal mass measuring at least 5.0 cm and were treated with curative intent were identified. A control group of 352 patients with nonmediastinal DLBL was selected for comparison.
The 43 patients with PMLBL had a male to female ratio of 20:23 and a median age of 42 years. Stage I/II disease was present in 58% of the patients, with only 9% having bone marrow involvement. A complete remission was achieved in 63% of the patients, and the 5-year overall and failure-free survivals were 46% and 38%, respectively. Among the clinical variables, an elevated serum lactate dehydrogenase level, a low performance score, more than one extranodal site, and an intermediate or high International Prognostic Index score were predictive of poor survival. When compared with the DLBL group, a younger median age was the only clinical feature that was significantly different in the PMLBL group.
The clinical features of PMLBL do not appear to be significantly different from those of nonmediastinal DLBL. Although the younger age of onset, slight female predominance, mediastinal location, and size of the mass may justify the recognition of PMLBL as a clinical syndrome, additional evidence is needed to define it as a distinct disease entity.
探讨原发性纵隔大B细胞淋巴瘤（PMLBL）是否为一种独特的临床病理实体，其病程是否比其他弥漫性大B细胞淋巴瘤（DLBL）更具侵袭性。
确定所有表现为纵隔肿块且直径至少5.0 cm、接受根治性治疗的CD20阳性DLBL患者。选择352例非纵隔DLBL患者作为对照组进行比较。
43例PMLBL患者的男女比例为20:23，中位年龄为42岁。58%的患者为Ⅰ/Ⅱ期疾病，仅9%有骨髓受累。63%的患者实现了完全缓解，5年总生存率和无失败生存率分别为46%和38%。在临床变量中，血清乳酸脱氢酶水平升高、体能状态评分低、一个以上结外部位以及国际预后指数评分为中或高是生存不良的预测因素。与DLBL组相比，PMLBL组唯一显著不同的临床特征是中位年龄较轻。
PMLBL的临床特征似乎与非纵隔DLBL没有显著差异。尽管发病年龄较轻、女性略占优势、纵隔位置和肿块大小可能使PMLBL被认为是一种临床综合征，但需要更多证据将其定义为一种独特的疾病实体。
