Miyamoto Ken-Ichi, Kobayashi Yukio, Maeshima Akiko Miyagi, Taniguchi Hirokazu, Nomoto Junko, Kitahara Hideaki, Fukuhara Suguru, Munakata Wataru, Maruyama Dai, Tobinai Kensei
Department of Hematology, National Cancer Center Hospital, Tsukiji 5-1-1, Chuo-ku, Tokyo, 104-0045, Japan.
Department of Pathology, National Cancer Center Hospital, Tokyo, Japan.
Int J Hematol. 2016 Jun;103(6):693-702. doi: 10.1007/s12185-016-1989-z. Epub 2016 Apr 19.
B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma (iBL/DLBCL), is a rare, but an aggressive subtype. In iBL/DLBCL, clinicopathological prognostic factors, including MYC and BCL2 translocations (double hit translocation, DHT) and the expression of both MYC and BCL2 (double hit score 2, DHS2), have not been studied thoroughly. We retrospectively analyzed the prognostic impact of clinicopathological factors, including MYC split, IGH/BCL2 fusion, MYC and BCL2 expressions, in 24 iBL/DLBCL patients (median age: 47 years). Fifteen patients (62 %) underwent intensive chemotherapy, and nine patients (38 %) underwent rituximab-cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP). The 5-year progression-free (PFS) and overall survival (OS) rates of intensive chemotherapy and R-CHOP were 57 and 72 %, respectively. PFS was significantly shorter in patients with high IPI score (P < .0001), stage IV (P = .001), aged ≥60 years (P = .042), IGH/BCL2 fusion (P = .029), DHS2 (P = .015), and DHT (P = .03). OS was significantly shorter in patients with high IPI score (P < .0001) and aged ≥60 years (P = .008). In iBL/DLBCL, IGH/BCL2 fusion, DHS2, and DHT were pathological prognostic factors for poor PFS, while IPI remained as more predictive for PFS and OS.
无法分类的B细胞淋巴瘤，具有弥漫性大B细胞淋巴瘤和伯基特淋巴瘤之间的中间特征（iBL/DLBCL），是一种罕见但侵袭性的亚型。在iBL/DLBCL中，包括MYC和BCL2易位（双打击易位，DHT）以及MYC和BCL2两者的表达（双打击评分2，DHS2）在内的临床病理预后因素尚未得到充分研究。我们回顾性分析了24例iBL/DLBCL患者（中位年龄：47岁）中包括MYC分裂、IGH/BCL2融合、MYC和BCL2表达在内的临床病理因素的预后影响。15例患者（62%）接受了强化化疗，9例患者（38%）接受了利妥昔单抗-环磷酰胺、阿霉素、长春新碱和泼尼松（R-CHOP）治疗。强化化疗和R-CHOP的5年无进展生存率（PFS）和总生存率（OS）分别为57%和72%。国际预后指数（IPI）评分高的患者（P<0.0001）、IV期患者（P=0.001）、年龄≥60岁的患者（P=0.042）、IGH/BCL2融合的患者（P=0.029）、DHS2的患者（P=0.015）和DHT的患者（P=0.03）的PFS明显较短。IPI评分高的患者（P<0.0001）和年龄≥60岁的患者（P=0.008）的OS明显较短。在iBL/DLBCL中，IGH/BCL2融合、DHS2和DHT是PFS不良的病理预后因素，而IPI对PFS和OS的预测性更强。
