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原发性纵隔大B细胞淋巴瘤:日本一项单机构临床研究

Primary mediastinal large B-cell lymphoma: a single-institution clinical study in Japan.

作者信息

Sekiguchi Naohiro, Nishimoto Junko, Tanimoto Kazuki, Kusumoto Shigeru, Onishi Yasushi, Watanabe Takashi, Kobayashi Yukio, Asamura Hisao, Kagami Yoshikazu, Matsuno Yoshihiro, Tobinai Kensei

机构信息

Hematology Division, National Cancer Center Hospital, Tokyo, Japan.

出版信息

Int J Hematol. 2004 Jun;79(5):465-71. doi: 10.1532/ijh97.03173.

Abstract

Several clinicopathologic studies of primary mediastinal large B-cell lymphoma (Med-DLBCL) have been reported from Western countries; however, only a few series of at most 10 cases are available in Japan. To further clarify the Med-DLBCL occurring in Japan, we analyzed the clinical features of 28 patients with Med-DLBCL diagnoses who were treated at the National Cancer Center Hospital between 1982 and 2002. The median age was 37 years (range, 18-80 years). The ages of 16 male patients ranged widely from 18 to 80 years, whereas the 12 female patients appeared to show a single age peak at 20 to 40 years. Only 13 patients (46%) achieved a complete response with initial treatments, mostly by CHOP-like regimens (cyclophosphamide, doxorubicin, vincristine [Oncovin], and prednisolone) followed by radiotherapy. The estimated 3-year overall and failure-free survival rates were 32% and 33%, respectively, indicating the relatively unfavorable prognosis of the patients in our series. The following factors were found to be significantly associated with shortened survival prospects: age >60 years, serum lactate dehydrogenase level greater than normal, performance status >1, and presence of bulky mediastinal mass. In conclusion, the clinical features of Japanese patients with Med-DLBCL may be different from those with the disease in Western countries. Because this investigation was a single-institution study with a limited number of patients, however, multicenter confirmatory studies are needed.

摘要

西方国家已经报道了几项关于原发性纵隔大B细胞淋巴瘤(Med-DLBCL)的临床病理研究;然而,在日本仅有少数系列报道,最多10例。为了进一步阐明日本发生的Med-DLBCL,我们分析了1982年至2002年间在国立癌症中心医院接受治疗的28例诊断为Med-DLBCL患者的临床特征。中位年龄为37岁(范围18 - 80岁)。16例男性患者年龄跨度较大,从18岁至80岁,而12例女性患者似乎在20至40岁出现一个年龄高峰。只有13例患者(46%)初始治疗获得完全缓解,大多采用类似CHOP的方案(环磷酰胺、阿霉素、长春新碱[安可平]和泼尼松龙)随后进行放疗。估计3年总生存率和无失败生存率分别为32%和33%,表明我们系列患者的预后相对较差。发现以下因素与生存前景缩短显著相关:年龄>60岁、血清乳酸脱氢酶水平高于正常、体能状态>1以及存在巨大纵隔肿块。总之,日本Med-DLBCL患者的临床特征可能与西方国家的患者不同。然而,由于本研究是一项单机构研究且患者数量有限,因此需要多中心验证性研究。

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