Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Department of Pathology, Microbiology, and Immunology, Vanderbilt University Medical Center, Nashville, TN, USA.
Histopathology. 2016 Jun;68(7):1090-8. doi: 10.1111/his.12884. Epub 2015 Dec 8.
Large B cell lymphomas with MYC and BCL6/3q27 rearrangements, designated MYC/BCL6 DHL, are uncommon. Our aim was to better characterize this group of tumours.
We studied the clinicopathological features and outcome of 13 patients with MYC/BCL6 DHL and compared this group to a group of 83 MYC/BCL2 DHL patients. There were eight men and five women, with a median age of 63 years. Eleven tumours were classified as diffuse large B cell lymphomas (DLBCL) and two were B cell lymphoma, unclassifiable, with features intermediate between DLBCL and Burkitt lymphoma (BCLU). Immunohistochemical analysis showed that these tumours were positive for BCL6 (100%), BCL2 (eight of 10; 80%) and CD10 (eight of 10; 80%). Nine of 12 (75%) cases had a germinal centre B cell (GCB) immunophenotype; in one case data were incomplete. All patients were treated with chemotherapy. The clinicopathological features of MYC/BCL6 DHL were similar to MYC/BCL2 DHL, except that MYC/BCL6 DHL had a GCB immunophenotype less often. Patients with MYC/BCL6 DHL had a poor overall survival, similar to patients with MYC/BCL2 DHL (P = 0.32).
MYC/BCL6 DHL is an aggressive B cell lymphoma and patients often have an aggressive clinical course and poor prognosis, similar to patients with MYC/BCL2 DHL.
具有 MYC 和 BCL6/3q27 重排的大 B 细胞淋巴瘤，称为 MYC/BCL6 DHL，并不常见。我们的目的是更好地描述这组肿瘤。
我们研究了 13 例 MYC/BCL6 DHL 患者的临床病理特征和结局，并将该组与 83 例 MYC/BCL2 DHL 患者进行了比较。其中男性 8 例，女性 5 例，中位年龄 63 岁。11 例肿瘤分类为弥漫性大 B 细胞淋巴瘤（DLBCL），2 例为 B 细胞淋巴瘤，无法分类，介于 DLBCL 和伯基特淋巴瘤（BCLU）之间。免疫组织化学分析显示这些肿瘤均为 BCL6（100%）、BCL2（10 例中的 8 例；80%）和 CD10（10 例中的 8 例；80%）阳性。9 例中有 12 例（75%）具有生发中心 B 细胞（GCB）免疫表型；1 例数据不完整。所有患者均接受化疗。除了 MYC/BCL6 DHL 患者的 GCB 免疫表型较少外，其临床病理特征与 MYC/BCL2 DHL 相似。MYC/BCL6 DHL 患者的总体生存率较差，与 MYC/BCL2 DHL 患者相似（P=0.32）。
MYC/BCL6 DHL 是一种侵袭性 B 细胞淋巴瘤，患者通常具有侵袭性的临床病程和较差的预后，与 MYC/BCL2 DHL 患者相似。
