邹鹤松, 刘薇, 张洪菊, 刘慧敏, 黄文阳, 安刚, 易树华, 邓书会, 隋伟薇, 王婷玉, 邱录贵, 邹德慧. 20例伴MYC BCL2和（或）BCL6重排的高级别B细胞淋巴瘤患者的临床特征及预后分析[J]. 中国肿瘤临床, 2022, 49(20): 1056-1061. DOI: 10.12354/j.issn.1000-8179.2022.20220343
引用本文: 邹鹤松, 刘薇, 张洪菊, 刘慧敏, 黄文阳, 安刚, 易树华, 邓书会, 隋伟薇, 王婷玉, 邱录贵, 邹德慧. 20例伴MYC BCL2和（或）BCL6重排的高级别B细胞淋巴瘤患者的临床特征及预后分析[J]. 中国肿瘤临床, 2022, 49(20): 1056-1061. DOI: 10.12354/j.issn.1000-8179.2022.20220343
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Hesong Zou, Wei Liu, Hongju Zhang, Huimin Liu, Wenyang Huang, Gang An, Shuhua Yi, Shuhui Deng, Weiwei Sui, Tingyu Wang, Lugui Qiu, Dehui Zou. Clinical characteristics and survival analysis of twenty patients with high-grade B-cell lymphoma with MYC, BCL2, and/or BCL6 rearrangements[J]. CHINESE JOURNAL OF CLINICAL ONCOLOGY, 2022, 49(20): 1056-1061. DOI: 10.12354/j.issn.1000-8179.2022.20220343
Citation: Hesong Zou, Wei Liu, Hongju Zhang, Huimin Liu, Wenyang Huang, Gang An, Shuhua Yi, Shuhui Deng, Weiwei Sui, Tingyu Wang, Lugui Qiu, Dehui Zou. Clinical characteristics and survival analysis of twenty patients with high-grade B-cell lymphoma with MYC, BCL2, and/or BCL6 rearrangements[J]. CHINESE JOURNAL OF CLINICAL ONCOLOGY, 2022, 49(20): 1056-1061. DOI: 10.12354/j.issn.1000-8179.2022.20220343
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20例伴MYC BCL2和（或）BCL6重排的高级别B细胞淋巴瘤患者的临床特征及预后分析

Clinical characteristics and survival analysis of twenty patients with high-grade B-cell lymphoma with MYC, BCL2, and/or BCL6 rearrangements

    摘要
  • 摘要:
      目的  分析伴MYC、BCL2和（或）BCL6重排的高级别B细胞淋巴瘤（high-grade B-cell lymphoma，HGBL-DH/TH）患者的临床特点、治疗及预后。
      方法  回顾性分析2015年1月至2020年11月在中国医学科学院血液病医院诊治的20例HGBL-DH/TH患者临床、病理资料及治疗转归。
      结果   20例患者中位年龄52（23～68）岁，男性占55%。75%患者Ann Arbor分期为Ⅲ～Ⅳ期，80%伴有结外侵犯，75%乳酸脱氢酶水平升高。所有患者诱导治疗最佳客观缓解率（objective response rate，ORR）和完全缓解率（complete response，CR）分别为75%和60%，中位随访27.7（3.8～74.6)个月，2年无进展生存（progression-free survival，PFS）率和总生存（overall survival，OS）率分别为55.0%和59.1%。局限期患者疗效优于进展期患者，两组最佳ORR分别为100.0%和61.5%，2年OS率分别为100.0%和45.7%。诱导治疗获得首次CR（first complete response, CR1）的12例患者中，除1例早期复发外均维持持续缓解状态（continuous complete response，CCR），其OS明显优于未达CR患者（P<0.001），2年OS率分别为91.7%和25.0%。复发或难治性(refractory/relapsed，R/R)患者预后极差，8例患者中仅1例挽救治疗获得CCR，其余7例患者均因疾病进展死亡。
      结论  本研究显示HGBL-DH/TH具有Ann Arbor分期晚、结外侵犯常见、LDH水平异常、总生存期短等特点。局限期相对进展期患者疗效更好。CR1患者生存良好，而R/R患者缺乏有效的挽救治疗，预后较差。

     

    Abstract:
      Objective  To evaluate the characteristics, treatments, and survival outcomes of patients with high-grade B-cell lymphoma (HGBL-DH/TH) with MYC, BCL2, and/or BCL6 rearrangements.
      Methods  Overall, 20 patients with HGBL-DH/TH who were diagnosed and treated at Institute of Hematology & Blood Diseases Hospital from January 2015 to November 2020 were included. Baseline characteristics, pathology findings, and survival outcomes were retrospectively analyzed.
      Results  The median age of the patients was 52(23–68) years, and 55% of patients were male. Fifteen (75%) patients had advanced Ann Arbor stage Ⅲ-Ⅳ HGBL, 16 (80%) had extranodal invasion, and 15 (75%) had increased lactate dehydrogenase (LDH) levels. The best objective response rate (ORR) and complete response (CR) rate in the entire cohort were 75% and 60%, respectively. The median follow-up duration was 27.7 (3.8–74.6) months, and the 2-year progression-free survival (PFS) and overall survival (OS) rates were 55.0% and 59.1%, respectively. The survival outcomes of patients with limited stage were better than those of patients with advanced stage. The best ORR in the two groups were 100% and 61.5%, and the 2-year OS rates were 100% and 45.7%, respectively. The 12 patients who achieved first complete response (CR1) after induction therapy maintained continuous complete response (CCR), except for one patient with early relapse, and their OS rate was significantly better than that of patients without CR (P<0.001). The 2-year OS rates were 91.7% and 25.0%, respectively; therefore, the prognosis of relapsed or refractory (R/R) patients was extremely poor, and seven patients died owing to disease progression, except for one patient who achieved CCR after salvage therapy.
      Conclusions   Patients with HGBL-DH/TH had the characteristics of advanced Ann Arbor stage, frequent extranodal invasion, abnormal LDH levels and short OS. The prognosis of patients with limited tumor stage was better than that of those with advanced stage. CR1 indicated a much better survival benefit, while patients with R/Rdisease lacked effective salvage therapies and had poor prognosis.

     

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