Kurita Naoki, Nishikii Hidekazu, Nakamoto Rie, Nakamura Naoya, Kondo Yuzuru, Okoshi Yasushi, Suzukawa Kazumi, Hasegawa Yuichi, Yokoyama Yasuhisa, Noguchi Masayuki, Chiba Shigeru
Department of Hematology, Graduate School of Comprehensive Human Sciences, University of Tsukuba, Ibaraki, Japan.
J Clin Exp Hematop. 2011;51(1):37-42. doi: 10.3960/jslrt.51.37.
B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma (iDLBCL/BL) is a newly categorized lymphoma in the WHO Classification of Tumors of Haematopoietic and Lymphoid Tissues v4. This lymphoma has characteristics of both DLBCL and BL, morphologically and genetically. We report a case with therapy-resistant iDLBCL/BL, which used to be categorized as BL in the WHO Classification v3. This case showed strong BCL2 staining, otherwise exhibiting features of BL. The case was treated with intensive chemotherapy containing high-dose methotrexate, cyclophosphamide, and cytarabine. However, soon after the chemotherapy, the lymphoma relapsed in the central nervous system and was resistant to whole-brain radiation therapy and allogeneic stem cell transplantation. The patient died 37 days after the transplantation. The clinical course was different from that of typical BL in terms of resistance to intensive chemotherapy, in agreement with the characteristics of iDLBCL/BL.
具有弥漫性大B细胞淋巴瘤和伯基特淋巴瘤之间特征的不可分类B细胞淋巴瘤（iDLBCL/BL）是《世界卫生组织造血与淋巴组织肿瘤分类》第4版中新分类的淋巴瘤。这种淋巴瘤在形态学和遗传学上具有弥漫性大B细胞淋巴瘤和伯基特淋巴瘤两者的特征。我们报告1例对治疗耐药的iDLBCL/BL病例，该病例在《世界卫生组织分类》第3版中曾被归类为伯基特淋巴瘤。该病例BCL2染色呈强阳性，其他方面表现为伯基特淋巴瘤的特征。该病例接受了包含大剂量甲氨蝶呤、环磷酰胺和阿糖胞苷的强化化疗。然而，化疗后不久，淋巴瘤在中枢神经系统复发，并且对全脑放射治疗和异基因干细胞移植耐药。患者在移植后37天死亡。就对强化化疗的耐药性而言，临床病程与典型伯基特淋巴瘤不同，这与iDLBCL/BL的特征相符。
