Clinicopathological features of double-hit B-cell lymphomas with MYC and BCL2, BCL6 or CCND1 rearrangements
Clinicopathological features of double-hit B-cell lymphomas with MYC and BCL2, BCL6 or CCND1 rearrangements
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DOI：
10.1111/pin.12335
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发表时间：
2015-10-01
影响因子：
2.2
通讯作者：
Ohshima, Koichi
中科院分区：
文献类型：
--
作者：
Yoshida, Maki;Ichikawa, Ayako;Ohshima, Koichi
双重打击（DH）淋巴瘤是以染色体重排为特征的B细胞淋巴瘤，特别是MYC和BCL 2、BCL 6或CCND 1。我们回顾了22例DH淋巴瘤。BCL 2/MYCDH淋巴瘤占DH淋巴瘤的大多数（17例; 77%），其次是BCL 6/MYC（2例; 9%）淋巴瘤。使用2008年世界卫生组织分类系统评估形态学特征，15例（68%）被确定为B细胞淋巴瘤，无法分类，其特征介于弥漫性大B细胞淋巴瘤（DLBCL）和伯基特淋巴瘤（BCLU）之间10例（45%），5例（23%）为DLBCL，2例（9%）为形态学未转化的滤泡性淋巴瘤。Burkitt淋巴瘤在DH淋巴瘤中少见（1例，5%）。19例患者接受R-CHOP或大剂量化疗方案治疗。中位随访11个月后，7例患者死亡，1年生存率为62.5%。大剂量化疗不能改善预后。我们建议，筛查遗传变异，以检测DH淋巴瘤是需要在诊断所有的淋巴瘤，即使是那些形态学确定为滤泡性淋巴瘤。
Double-hit (DH) lymphomas are B-cell lymphomas characterized by chromosomal rearrangements, specifically of MYC and either BCL2, BCL6 or CCND1. We reviewed 22 cases of DH lymphomas. BCL2/MYCDH lymphomas constituted the majority of these DH lymphomas (17 cases; 77%), followed by BCL6/MYC (2 cases; 9%) lymphomas. Assessing morphological features using the 2008 World Health Organization classification system, 15 cases (68%) were determined to be B-cell lymphoma, unclassifiable with features intermediate between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma (BCLU) (10 cases; 45%), or as DLBCL (5 cases; 23%), and 2 cases (9%) were classified as morphologically untransformed follicular lymphoma. Burkitt lymphoma was rare (1 case; 5%) among DH lymphomas. Nineteen cases were treated with R-CHOP or a high dose chemotherapy regimen. After a median follow-up of 11 months, 7 patients had died, and the 1-year survival rate was 62.5%. High dose chemotherapy did not improve the outcome. We suggest that screening of genetic variations to detect DH lymphomas is required in diagnosing all lymphomas, even those determined morphologically to be follicular lymphoma.