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原发性纵隔大B细胞淋巴瘤:韩国单中心经验

Primary mediastinal large B-cell lymphoma: a single-center experience in Korea.

作者信息

Ahn Heui June, Yoon Dok-Hyun, Kim Shin, Lee Kyoungmin, Kang Eunhee, Huh Jooryung, Park Chan-Sik, Suh Cheolwon

机构信息

Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea. ; Department of Internal Medicine, Gangneung Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

出版信息

Blood Res. 2014 Mar;49(1):36-41. doi: 10.5045/br.2014.49.1.36. Epub 2014 Mar 24.

Abstract

BACKGROUND

Primary mediastinal large B-cell lymphoma (PMBL) is a distinct subtype of non-Hodgkin lymphoma, which has no consensus for its ideal treatment or prognosis.

METHODS

We reviewed the clinicopathologic features and clinical outcomes of 25 PMBL cases diagnosed at a single institution between 1993 and 2009 and compared them with 588 cases of non-mediastinal, diffuse large B-cell lymphoma (DLBCL, control group) diagnosed during the same period.

RESULTS

Thirteen (52.0%) PMBL patients had Ann Arbor stage III or IV disease, and 10 (40.0%) had B symptoms. Thirteen (52%) PMBL patients were classified as high-intermediate/high-risk according to the International Prognostic Index. There was a significant prevalence of young (median: 31 years; range, 15-78 years; P<0.001), female (68%; P=0.014) patients in the PMBL group compared to the control group (median: 56 years; range, 15-85 years; 43.2% female). Bulky disease and elevated levels of lactate dehydrogenase (LDH) were more frequent in the PMBL group (P<0.001 and P=0.003, respectively). Nineteen (76%) PBML patients achieved complete remission, and 18 were alive at the last follow-up (median: 43 months; range, 1-92 months). There was no difference in the 3-year, overall survival rate (72%, 95% confidence interval [CI]: 54.0-83.0 versus 70.1%, 95% CI, 109.0-126.0; P=0.686) between PMBL and control patients, respectively.

CONCLUSION

Compared to patients with non-mediastinal DLBCL, Korean patients with PMBL are predominantly young women with bulky disease and high LDH levels but with no significant difference in survival.

摘要

背景

原发性纵隔大B细胞淋巴瘤(PMBL)是非霍奇金淋巴瘤的一种独特亚型,其理想治疗方案或预后尚无共识。

方法

我们回顾了1993年至2009年间在单一机构诊断的25例PMBL病例的临床病理特征和临床结局,并将其与同期诊断的588例非纵隔弥漫性大B细胞淋巴瘤(DLBCL,对照组)病例进行比较。

结果

13例(52.0%)PMBL患者Ann Arbor分期为III或IV期,10例(40.0%)有B症状。根据国际预后指数,13例(52%)PMBL患者被归类为高中间/高危。与对照组(中位年龄:56岁;范围,15 - 85岁;女性占43.2%)相比,PMBL组年轻患者(中位年龄:31岁;范围,15 - 78岁;P < 0.001)和女性患者(68%;P = 0.014)的患病率显著更高。PMBL组中大包块病变和乳酸脱氢酶(LDH)水平升高更为常见(分别为P < 0.001和P = 0.003)。19例(76%)PBML患者实现完全缓解,18例在最后一次随访时存活(中位时间:43个月;范围,1 - 92个月)。PMBL患者和对照患者的3年总生存率分别为72%(95%置信区间[CI]:54.0 - 83.0)和70.1%(95% CI,109.0 - 126.0),无差异(P = 0.686)。

结论

与非纵隔DLBCL患者相比,韩国PMBL患者主要是患有大包块病变和LDH水平高的年轻女性,但生存率无显著差异。

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