Kanungo Anuradha, Medeiros L Jeffrey, Abruzzo Lynne V, Lin Pei
Department of Hematopathology, The University of Texas, MD Anderson Cancer Center, Houston, TX 77030, USA.
Mod Pathol. 2006 Jan;19(1):25-33. doi: 10.1038/modpathol.3800500.
We identified 14 B-cell neoplasms with concurrent t(14;18) and chromosome 8q24 or c-MYC translocations shown by conventional cytogenetics or fluorescence in situ hybridization analysis. All cases assessed by conventional cytogenetics had a complex karyotype. There were 10 men and four women, with a median age of 55 years (range, 29-72). None of these patients had a history of follicular lymphoma. The biopsy specimens were obtained from bone marrow, lymph node, and extranodal sites. Morphologically, nine neoplasms had features of Burkitt or atypical Burkitt lymphoma/leukemia and three were diffuse large B-cell lymphoma with high-grade cytologic features. The remaining two cases were plasmablastic myeloma and low-grade B-cell lymphoma, respectively. All cases expressed BCL-2. The proliferation index assessed by using Ki-67 (MIB1) was 5% in the low-grade B-cell lymphoma, 80% in the plasmablastic myeloma, 90-95% in three cases of diffuse large B-cell lymphoma, and ranged from 90 to >99% in most Burkitt and atypical Burkitt neoplasms. The patient with low-grade B-cell lymphoma was treated with rituximab. All other patients received intensive combination chemotherapy. Two of these patients underwent bone marrow transplantation, and one patient received radiation therapy in addition to transplantation. The median follow-up period was 9 months (range, 3-81). In all, 10 patients died with a median survival of 9 months (range, 3-81). We conclude that most B-cell lymphomas with concurrent t(14;18) and 8q24/c-MYC translocations fall within the morphologic spectrum of diffuse large B-cell and Burkitt lymphoma. These neoplasms are high-grade and are associated with a poor prognosis. However, this combination of molecular abnormalities can also rarely occur in other neoplasms, such as the cases of low-grade B-cell lymphoma and plasmablastic myeloma in this study.
我们通过传统细胞遗传学或荧光原位杂交分析，鉴定出14例同时存在t(14;18)以及8号染色体q24或c-MYC易位的B细胞肿瘤。所有经传统细胞遗传学评估的病例均具有复杂核型。患者中男性10例，女性4例，中位年龄55岁（范围29 - 72岁）。这些患者均无滤泡性淋巴瘤病史。活检标本取自骨髓、淋巴结及结外部位。形态学上，9例肿瘤具有伯基特或非典型伯基特淋巴瘤/白血病特征，3例为具有高级别细胞学特征的弥漫性大B细胞淋巴瘤。其余2例分别为浆母细胞性骨髓瘤和低级别B细胞淋巴瘤。所有病例均表达BCL-2。使用Ki-67（MIB1）评估的增殖指数在低级别B细胞淋巴瘤中为5%，在浆母细胞性骨髓瘤中为80%，在3例弥漫性大B细胞淋巴瘤中为90% - 95%，在大多数伯基特和非典型伯基特肿瘤中为90%至>99%。低级别B细胞淋巴瘤患者接受了利妥昔单抗治疗。所有其他患者接受了强化联合化疗。其中2例患者接受了骨髓移植，1例患者除移植外还接受了放射治疗。中位随访期为9个月（范围3 - 81个月）。总共有10例患者死亡，中位生存期为9个月（范围3 - 81个月）。我们得出结论，大多数同时存在t(14;18)和8q24/c-MYC易位的B细胞淋巴瘤属于弥漫性大B细胞和伯基特淋巴瘤的形态学范畴。这些肿瘤为高级别，且预后不良。然而，这种分子异常的组合也很少见于其他肿瘤，如本研究中的低级别B细胞淋巴瘤和浆母细胞性骨髓瘤病例。
