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双打击淋巴瘤在利妥昔单抗时代构成弥漫性大 B 细胞淋巴瘤中极具侵袭性的亚组。

Double-hit lymphomas constitute a highly aggressive subgroup in diffuse large B-cell lymphomas in the era of rituximab.

机构信息

*Division of Hematology and Oncology, Department of Medicine, Kyoto Prefectural University of Medicine, and Department of Hematology, Kyoto First Red Cross Hospital, 465 Kajii-cho, Kamigyo-ku, Kyoto 602-8566, Japan.

出版信息

Jpn J Clin Oncol. 2012 Nov;42(11):1035-42. doi: 10.1093/jjco/hys148. Epub 2012 Sep 14.

DOI:10.1093/jjco/hys148
PMID:22984125
Abstract

OBJECTIVE

The incorporation of rituximab in immunochemotherapy has improved treatment outcomes for diffuse large B-cell lymphoma, but the prognosis for some diffuse large B-cell lymphomas remains dismal. Identification of adverse prognostic subgroups is essential for the choice of appropriate therapeutic strategy.

METHODS

We retrospectively investigated the impact of so-called 'double-hit' cytogenetic abnormalities, i.e. cytogenetic abnormalities involving c-MYC co-existing with other poor prognostic cytogenetic abnormalities involving BCL2, BCL6 or BACH2, on treatment outcomes for 93 consecutive diffuse large B-cell lymphoma patients.

RESULTS

According to the revised international prognostic index, no patients were cytogenetically diagnosed with double-hit lymphomas in the 'very good' risk group or in the 'good' risk group, while 5 of 33 patients had double-hit lymphomas in the 'poor' risk group. All the double-hit lymphoma patients possessed both nodal and extranodal involvement. The overall complete response rate was 89.3%, overall survival 87.1% and progression-free survival 75.8% over 2 years (median observation period: 644 days). The complete response rates were 93.2% for the non-double-hit lymphoma patients and 40.0% for the double-hit lymphoma patients. Significantly longer progression-free survival and overall survival were observed for the 'very good' and the 'good' risk patients than for the 'poor' risk patients. Moreover, the progression-free survival of double-hit lymphoma was significantly shorter than that of the non-double-hit lymphoma 'poor' risk patients (P = 0.016). In addition, the overall survival of the double-hit lymphoma patients also tended to be shorter than that of the non-double-hit lymphoma 'poor' risk group.

CONCLUSIONS

The diagnosis of double-hit lymphoma can help discriminate a subgroup of highly aggressive diffuse large B-cell lymphomas and indicate the need for the development of novel therapeutic strategies for double-hit lymphoma.

摘要

目的

利妥昔单抗联合免疫化疗改善了弥漫性大 B 细胞淋巴瘤的治疗效果,但某些弥漫性大 B 细胞淋巴瘤的预后仍然较差。确定不良预后亚组对于选择适当的治疗策略至关重要。

方法

我们回顾性研究了所谓的“双打击”细胞遗传学异常,即涉及 c-MYC 的细胞遗传学异常与涉及 BCL2、BCL6 或 BACH2 的其他不良预后细胞遗传学异常共存,对 93 例连续弥漫性大 B 细胞淋巴瘤患者的治疗结果的影响。

结果

根据修订的国际预后指数,在“非常好”风险组或“良好”风险组中,没有患者被诊断为双打击淋巴瘤,而在“不良”风险组中有 5 例患者被诊断为双打击淋巴瘤。所有双打击淋巴瘤患者均有结内和结外累及。2 年内的总完全缓解率为 89.3%,总生存率为 87.1%,无进展生存率为 75.8%(中位观察期:644 天)。非双打击淋巴瘤患者的完全缓解率为 93.2%,双打击淋巴瘤患者的完全缓解率为 40.0%。“非常好”和“良好”风险患者的无进展生存和总生存明显长于“不良”风险患者。此外,双打击淋巴瘤的无进展生存明显短于非双打击淋巴瘤“不良”风险患者(P = 0.016)。此外,双打击淋巴瘤患者的总生存也倾向于短于非双打击淋巴瘤“不良”风险组。

结论

双打击淋巴瘤的诊断有助于区分高度侵袭性弥漫性大 B 细胞淋巴瘤亚组,并表明需要为双打击淋巴瘤制定新的治疗策略。

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