Yoshida Maki, Ichikawa Ayako, Miyoshi Hiroaki, Kiyasu Junichi, Kimura Yoshizo, Arakawa Fumiko, Niino Daisuke, Ohshima Koichi
Department of Pathology, School of Medicine, Kurume University, Kurume, Fukuoka, Japan.
Pathol Int. 2015 Oct;65(10):519-27. doi: 10.1111/pin.12335. Epub 2015 Jul 29.
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/MYC DH 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.
双打击（DH）淋巴瘤是一种B细胞淋巴瘤，其特征是存在染色体重排，特别是MYC与BCL2、BCL6或CCND1之一的重排。我们回顾了22例DH淋巴瘤病例。BCL2/MYC DH淋巴瘤占这些DH淋巴瘤的大多数（17例；77%），其次是BCL6/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%），或为DLBCL（5例；23%），2例（9%）被分类为形态学上未转化的滤泡性淋巴瘤。在DH淋巴瘤中，伯基特淋巴瘤很少见（1例；5%）。19例患者接受了R-CHOP或高剂量化疗方案治疗。中位随访11个月后，7例患者死亡，1年生存率为62.5%。高剂量化疗并未改善预后。我们建议，在诊断所有淋巴瘤时，即使是那些形态学上确定为滤泡性淋巴瘤的病例，也需要进行基因变异筛查以检测DH淋巴瘤。
